Atlas of the Newborn [Vol 4] - A. Rudolph (BC Decker, 1997) WW

Rudolph

NewbornAtlas of the

V O L U M E 4

Dermatology and Perinatal Infection

V O L U M E 4


NewbornAtlas of the

Arnold J. Rudolph, M.D.1918–1995

Professor of Pediatrics,Obstetrics and GynecologyBaylor College of Medicine

Houston, Texas

V O L U M E 4


Arnold J. Rudolph, M.D. 1918-1995

Professor of Pediatrics Baylor Medical College

Houston, Texas

1997

B.C. Decker Inc. Hamilton • London

NewbornAtlas of the

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ForewordSir William Osler stated, “There is no more

difficult task in medicine than the art ofobservation.” The late Arnold Jack Rudolphwas an internationally renowned neonatolo-gist, a teacher’s teacher, and, above all, onewho constantly reminded us about how muchcould be learned by simply observing, in hiscase, the newborn infant.

This color atlas of neonatology represents adistillation of more than 50 years of observingnormal and abnormal newborn infants. TheAtlas begins with a section on the placenta,its membranes, and the umbilical cord. JackRudolph delighted in giving a lecture entitled“Don’t Make Mirth of the Afterbirth,” inwhich he captivated audiences by showingthem how much you could learn about thenewborn infant from simply observing theplacenta, its membranes, and the umbilicalcord.

In a few more than 60 photomicrographs,we learn to read the placenta and gain insightinto such disorders as intrauterine growthretardation, omphalitis, cytomegalic inclu-sion disease, congenital syphilis, and congen-ital neuroblastoma. Congenital abnormalitiesof every organ system are depicted along withthe appearance of newborn infants who havebeen subjected in utero to a variety of differ-ent drugs, toxins, or chemicals. We also learnto appreciate the manifestations of birth trau-ma and abnormalities caused by abnormalintrauterine positioning.

More than 250 photographs are used toillustrate the field of neonatal dermatology.The collection of photographs used in thissection is superior to that which I have seenin any other textbook or atlas of neonatologyor dermatology; this section alone makes thisreference a required addition to the library ofany clinician interested in the care of infantsand children. Photographs of the Kasabach-Merritt syndrome (cavernous hemangiomawith thrombocytopenia), Klippel-Trenaunaysyndrome, Turner’s syndrome, Waardenburg’ssyndrome, neurocutaneous melanosis, mas-tocytosis (urticaria pigmentosa), and incon-

tinentia pigmenti (Bloch-Sulzberger syn-drome) are among the best that I have seen.

Cutaneous manifestations are associatedwith many perinatal infections. The variedmanifestations of staphylococcal infection ofthe newborn are depicted vividly in photomi-crographs of furunculosis, pyoderma, bullousimpetigo, abscesses, parotitis, dacryocystitis,inastitis, cellulitis, omphalitis, and funisitis.Streptococcal cellulitis, Haemophilus influen-zae cellulitis, and cutaneous manifestations oflisteriosis all are depicted. There are numer-ous photomicrographs of congenital syphilis,showing the typical peripheral desquamativerash on the palms and soles, as well as otherpotential skin manifestations of congenitalsyphilis which may produce either vesicular,bullous, or ulcerative lesions. The variousradiologic manifestations of congenitalsyphilis, including pneumonia alba, ascites,growth arrest lines, Wegner’s sign, periostitis,and syphilitic osteochondritis, are depicted.Periostitis of the clavicle (Higouménaki’ssign) is shown in a photograph that alsodepicts periostitis of the ribs. A beautiful pho-tomicrograph of Wimberger’s sign also hasbeen included; this sign, which may appear inan infant with congenital syphilis, revealsradiolucency due to erosion of the medialaspect of the proximal tibial metaphysis.

The Atlas also includes a beautiful set ofphotographs which delineate the ophthalmo-logic examination of the newborn. Lesionswhich may result from trauma, infection, orcongenital abnormalities are included. Thereare numerous photographs of the ocular man-ifestations of a variety of systemic diseases,such as Tay-Sachs disease, tuberous sclerosis,tyrosinase deficiency, and many more.Photographs of disturbances of each of thevarious organ systems, or disorders affectingsuch organ systems, also are included alongwith numerous photographs of different formsof dwarfism, nonchromosomal syndromes andassociations, and chromosomal disorders. Inshort, this Atlas is the complete visualtextbook of neonatology and will provide any

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physician, nurse, or student with a distillationof 50 years of neonatal experience as viewedthrough the eyes of a master clinician.

Arnold Jack Rudolph was born in 1918,grew up in South Africa, and graduated fromthe Witwatersrand Medical School in 1940.Following residency training in pediatrics atthe Transvaal Memorial Hospital forChildren, he entered private pediatric prac-tice in Johannesburg, South Africa. Afteralmost a decade, he left South Africa andmoved to Boston, where he served as a SeniorAssistant Resident in Medicine at theChildren’s Medical Center in Boston,Massachusetts, and subsequently pursued fel-lowship training in neonatology at the sameinstitution and at the Boston Lying-InHospital, Children’s Medical Center andHarvard Medical School under Dr. ClementA. Smith.

In 1961, Dr. Rudolph came to BaylorCollege of Medicine in Houston, Texas, theschool at which he spent the remainder of hiscareer. He was a master teacher, who receivedthe outstanding teacher award from pediatricmedical students on so many occasions thathe was elected to the Outstanding FacultyHall of Fame in 1982. Dr. Rudolph alsoreceived numerous awards over the years fromthe pediatric house staffs for his superb teach-ing skills.

He was the Director of the NewbornSection in the Department of Pediatrics atBaylor College of Medicine for many years,until he voluntarily relinquished that posi-tion in 1986 for reasons related to his health.

Nevertheless, Jack Rudolph continued towork extraordinarily long hours in the care ofthe newborn infant, and was at the bedsideteaching both students and house staff, aswell as his colleagues, on a daily basis untiljust a few months before his death in July1995.

Although Dr. Rudolph was the author orco-author of more than 100 published papersthat appeared in the peer-reviewed medicalliterature, his most lasting contribution toneonatology and to pediatrics is in the legacyof the numerous medical students, house staff,fellows, and other colleagues whom he taughtincessantly about how much one could learnfrom simply observing the newborn infant.This Atlas is a tour de force; it is a spectacularteaching tool that has been developed, collat-ed, and presented by one of the finest clinicalneonatologists in the history of medicine. It isan intensely personal volume that, as Dr.Rudolph himself states, “is not intended torival standard neonatology texts,” but ratherto supplement them. This statement revealsDr. Rudolph’s innate modesty, since with theexception of some discussion on pathogenesisand treatment, it surpasses most neonatologytexts in the wealth of clinical informationthat one can derive from viewing and imbib-ing its contents. We owe Dr. Rudolph andthose who aided him in this work a debt ofgratitude for making available to the medicalcommunity an unparalleled visual referenceon the normal and abnormal newborn infant.

Ralph D. Feigin, M.D.June 13, 1996

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PrefaceI first became attracted to the idea of pro-

ducing a color atlas of neonatology manyyears ago. However, the impetus to synthesizemy experience and compile this current col-lection was inspired by the frequent requestsfrom medical students, pediatric house staff,nurses and others to provide them with acolor atlas of the clinical material provided inmy “slide shows.” For the past few decades Ihave used the medium of color slides and radiographs as a teaching tool. In these week-ly “slide shows” the normal and abnormal, aswords never can, are illustrated.

“I cannot define an elephant but I know onewhen I see one.”1

The collection of material used has beenadded to constantly with the support of thepediatric house staff who inform me to “bringyour camera” whenever they see an unusualclinical finding or syndrome in the nurseries.

A thorough routine neonatal examination isthe inalienable right of every infant. Mostnewborn babies are healthy and only a rela-tively small number may require special care.It is important to have the ability to distin-guish normal variations and minor findingsfrom the subtle early signs of problems. Thetheme that recurs most often is that carefulclinical assessment, in the traditional sense, isthe prerequisite and the essential foundationfor understanding the disorders of the new-born. It requires familiarity with the widerange of normal, as well as dermatologic, car-diac, pulmonary, gastrointestinal, genitouri-nary, neurologic, and musculoskeletal disor-ders, genetics and syndromes. A background in general pediatrics and a working knowl-edge of obstetrics are essential. The generallayout of the atlas is based on the above.Diseases are assigned to each section on thebasis of the most frequent and obvious pre-senting sign. It seems probable that the find-ings depicted will change significantly in the

decades to come. In this way duplication hasbeen kept to a minimum. Additional spacehas been devoted to those areas of neonatalpathology (e.g., examination of the placenta,multiple births and iatrogenesis) which poseparticular problems or cause clinical concern.

Obviously, because of limitations of space,it is impossible to be comprehensive andinclude every rare disorder or syndrome. Ihave tried to select both typical findings andvariations in normal infants and those foundin uncommon conditions. Some relevantconditions where individual variations needto be demonstrated are shown in more thanone case.

As the present volume is essentially one ofmy personal experience, it is not intended torival standard neonatology texts, but is pre-sented as a supplement to them. It seemslogical that references should be to standardtexts or reviews where discussion on patho-genesis, treatment, and references to originalworks may be found.

Helen Mintz Hittner, M.D., has been kindenough to contribute the outstanding sectionon neonatal ophthalmology.

I have done my best to make the necessaryacknowledgements to the various sources forthe clinical material. If I have inadvertentlyomitted any of those, I apologize. My mostsincere appreciation and thanks to DonnaHamburg, M.D., Kru Ferry, M.D., MichaelGomez, M.D., Virginia Schneider, PA, andJeff Murray, M.D., who have spent in-numerable hours in organizing and culling thematerial from my large collection. We wish tothank Abraham M. Rudolph, M.D., for hisassistance in reviewing the material. We alsowish to thank the following people for theirphotographic contributions to this work:Gerardo Cabrera-Meza, Morven Edwards,John Kenny, Claire Langston, Moise Levy,Ken Moise and Don Singer.

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It is hoped that this atlas will provideneonatologists, pediatricians, family physi-cians, medical students and nurses with a basisfor recognizing a broad spectrum of normalvariations and clinical problems as well asprovide them with an overall perspective ofneonatology, a field in which there continuesto be a rapid acceleration of knowledge and

technology. One must bear in mind thecaveat that pictures cannot supplant clinicalexperience in mastering the skill of visualrecall.

1. Senile dementia of Alzheimer’s type — normal aging ordisease? (Editorial) Lancet 1989; i:476-477.

Arnold J. Rudolph, M.D.

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CONTENTS

Volume 4Dermatology and Perinatal Infection

1. Neonatal Dermatology 1

2. Perinatal Infection 87

Index 147

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Although several texts provide extensive written descriptions of the newborn infant, thesenses of touch, hearing, and especially sight, create the most lasting impressions. Over aperiod of almost five decades, my brother Jack Rudolph diligently recorded, in pictorial form,his vast experiences in physical examination of the newborn infant. Atlas of the Newbornreflects his selection from the thousands of color slides in his collection, and truly representsthe “art of medicine” as applied to neonatology. A number of unusual or rare conditions areincluded in this atlas. I consider this fully justified, because if one has not seen or heard of acondition, one will never be able to diagnose it.

This fourth volume of the five-volume series reviews two main areas; disorders of the skin,and perinatal infections.

Clinical assessment of skin disorders in the newborn infant is one of the most challengingproblems confronting neonatologists and pediatricians, and many feel quite insecure inevaluating skin lesions in neonates. Chapter 1, Dermatology, provides a unique and elegantcollection of color photographs illustrating many of the skin abnormalities that may beencountered in the newborn. The section depicting benign and transient skin lesions is ofparticular interest, and congenital abnormalities, vascular malformations, disorders ofpigmentation, vesiculo-bullous lesions, and scaling conditions are extensively reviewed.This is perhaps the most detailed graphic presentation of dermatologic problems in thenewborn infant currently available.

Chapter 2, Perinatal Infection, provides excellent illustrations of various organ systeminvolvements in the neonate resulting from infection by bacteria and by syphilis, as well as prenatal viral infections such as cytomegalovirus, rubella, and varicella, and infection by protozoa such as toxoplasma. The section depicting congenital syphilis is especially com-prehensive, covering the protean manifestations of this infection in the infant. The effects of neonatally-acquired infections due to bacteria, herpes virus and fungi are also elegantlyillustrated.

Volume IV of Atlas of the Newborn will be of enormous value to general pediatricians,neonatologists, obstetricians and nurses involved in perinatal care, and also to dermatologistsand infectious disease specialists.

Abraham M. Rudolph, M.D.

Introduction

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Chapter 1Neonatal DermatologyThe neonatal skin must be given careful consideration for several reasons:

- It is a protective organ, especially when covered with vernix.- Any break in integrity creates an opportunity for infection; therefore, minimize skin trauma.- Absorption of agents through the skin, especially in premature infants, may have harmful

effects (e.g., hexachlorophene, Betadine™, boric acid, etc.)- The skin may be used therapeutically (e.g., application of safflower oil for essential fatty acid

deficiency).

Neonatal skin may present a bewildering variety of lesions; some innocent, temporary and theresult of a physiologic response; others the result of an episodic disease; and still others indicativeof a serious, often fatal, underlying disorder.Dermatologic manifestations of infection are presented in Chapter 2, Perinatal Infection.

2 Dermatology and Perinatal Infection

Figure 1.1. In this infant there is a regular segment-likepattern of transverse folding creases across the lower tho-rax and abdomen giving the trunk a “gridiron” appear-ance. Note the prominent linea nigra. This finding ismore common in postmature infants.

Figure 1.2. Pigmentation of theareola of the nipple in a newborninfant. Pigmentation is moremarked in black infants.

Figure 1.3. Pigmentation at the base of the nails in a blackinfant. There may be little pigmentation of the skin in general atbirth, but the finding of pigmentation at the base of the nails,pinnae of the ears, axilla, areolae of the nipples, genitalia, and aprominent linea nigra would all suggest that the infant is a blackinfant.

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BENIGN AND TRANSIENT CUTANEOUS LESIONS

These lesions are commonly observed in a normal nursery population, and none require special therapeuticconsideration. Numerous benign minor variations in the skin noted in the routine care of babies who are wellinclude skin pigmentation, desquamation, vernix caseosa, etc.

Figure 1.4. Linea nigra andpigmentation of the skin and gen-italia in a black female infant.Linea nigra is the line of increasedpigmentation extending from theumbilicus to the more darkly pig-mented genitalia. This area ofbenign pigmentation becomes lessprominent as the baby’s skin dark-ens. In this postmature infant notethe area of lack of pigmentation inboth groins.

Figure 1.5. The same infant as inFigure 1.4 with her thighsadducted. Note that the areaslacking pigmentation in thegroins correspond to the area pro-tected in utero by adduction ofthe lower limbs.

Figure 1.6. Vernix caseosa pre-sent in a term infant at birth. Thevernix caseosa is a fetal product ofthe sebaceous glands, shed epithe-lial cells, and hair. It is cheesy-white in appearance and may beliberally caked all over the skin orconcentrated in folds like thegroins and genitalia, the axilla, orbehind the ears. Its presence sug-gests that the infant is close toterm as the vernix disappears withincreasing gestational age. Thevernix normally is white in colorbut can alter to a golden-yellow ininfants that are meconium-stainedfrom fetal distress or in infantswith erythroblastosis fetalis.

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Neonatal Dermatology 3


Figure 1.7. A minimal amount ofvernix caseosa in the groins andgenitalia of a male infant.

Figure 1.8. Vernix caseosa in afemale infant. After birth theinfant is bathed and the vernixremoved by the nurse. If there isany question as to whether or notthere was any meconium staining,certain areas such as the axilla,inguinal folds and genitalia, if notadequately cleaned, will revealtraces of the vernix.

Figure 1.9. Meconium staining ofthe vernix and skin in an infantwho had fetal distress with meco-nium-stained amniotic fluid.

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Figure 1.10. Note the meconium staining ofthe skin in a post-term infant. He also hadmarked desquamation of the skin as well as longfinger nails, features common to the postmatureinfant.

Figure 1.11. The hand of the same infant as inFigure 1.1. showing the desquamation of theskin, long finger nails, and meconium staining.

Figure 1.12. Marked desquamationof the skin in a post-term infant. Thisis a benign physiologic desquamationwith paper-thin peeling; the underly-ing skin is normal. It occurs withpostmaturity as the vernix disappearsand the skin of the fetus is not pro-tected from the amniotic fluid. Theprocess is more marked in areas ofirritation.

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Figure 1.13. Desquamation ofthe skin is a very typical findingin an abdominal pregnancy inwhich the fetus is not protectedby the amniotic fluid.

Figure 1.14. Hypertrichosis(hirsutism) is very common innormal mature Hispanic infants.

Figure 1.15. Another infantwith hypertrichosis over thebody at birth. Note thatalthough this is very striking it isa normal finding in normalmature Hispanic infants.

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Figure 1.16. Acrocyanosis in an otherwise normalinfant. Peripheral vasoconstriction occurs very com-monly in normal infants and rapidly improves whenthe infant is warmed or cries.

Figure 1.17. In this infant notethe maculopapular rash whichfollowed phototherapy treatmentfor hyperbilirubinemia. This“bilirubin rash” improves rapidlyfollowing discontinuation ofphototherapy.

Figure 1.18. Mongolian spots in a caucasianinfant. Mongolian spots are a minor anomalycommonly found in infants of darkly pigmentedracial groups. They occur in 5 to 10% of cau-casian infants, 70% of hispanic and orientalinfants, and over 90% of black infants. The cir-cumscribed bluish-grey to dark blue areas of discoloration are usually found over the lum-bosacral area and lower back, rarely extending asfar as the shoulders and neck, and can also befound on the limbs. They tend to be on theouter surfaces of the body when the infant isplaced in its fetal position and are due to anaccumulation of dopa-positive melanocytes.

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Figure 1.19. Mongolian spots ina black infant. The spots have nosignificance but are sometimesmistaken for bruises, causing asuspicion of child abuse. Thisshould be kept in mind whenintentional injury is questioned.They fade during childhood orappear to fade as the skin dark-ens. As mongolian spots arenever elevated and are not palpa-ble, they can be differentiatedfrom a blue nevus which is raisedand is located on the arms, legs,or face and persists throughoutlife.

Figure 1.20. Cutis marmorata isa common finding in normalinfants. This fine reticulatedmottled appearance is due tovasomotor instability and thus ismore commonly seen in prema-ture infants, but should also alertone to the possibility of sepsis,hypothyroidism, and central ner-vous system pathology.

Figure 1.21. In the harlequinsign (harlequin color change)there is a vivid line of demarca-tion which appears down themidline. The dependent side of the skin becomes flushed (erythematous) and the upper-most side becomes pale. If theinfant is turned to the other side,the appearance of the skinreverses. It is proposed that thiscondition results from vasomotorinstability.

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Figure 1.22. The harlequin sign inanother infant showing the frontaland posterior views. This conditionoccurs most commonly in prema-ture infants, is rare in term infants,and is of no pathologic signifi-cance. It may recur repeatedly inthe same infant but disappearswithin the first few months of life.Harlequin sign is not to be be con-fused with the harlequin fetus(ichthyosis congenita).

Figure 1.23. Erythema toxicumneonatorum (urticaria neonato-rum) on the back of a term infant.This is the most common rashnoted in the normal term infant. Itis not seen in preterm and rarelyseen in post-term infants. It usuallyappears on the 2nd or 3rd day oflife (rarely in the first 24 hours)and is seldom seen after the age of14 days. It affects about 40 to 50%of full term infants and the condi-tion is self-limiting. Lesions may beminimal or extensive.

Figure 1.24. Another example oferythema toxicum neonatorum(“flea bite” dermatitis of the new-born). The lesions most frequentlypresent are erythematous and mac-ulopapular, but macules or papulesmay predominate. The lesionscome and go on various sites on thetrunk and limbs before they disap-pear permanently. The rash maybecome confluent and intensifiedin areas subject to irritation.

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Figure 1.25. Close-up of the lesions in erythema toxicum neonatorum.The etiology is unknown but biopsy of the lesions show the presence ofnumerous eosinophils. It has been suggested that the presence of erythematoxicum is evidence of maturity.

Figure 1.26. Erythema toxicumneonatorum may present asvesicular lesions, which werepresent at birth in this infant.Note the lack of inflammationsurrounding the vesicles (whichactually look like pustules). Theselesions may be associated withthe more usual maculopapularlesions elsewhere on the skin.The diagnosis of erythema toxi-cum was confirmed by examina-tion of a stained smear from thelesions in which eosinophils alonewere present and numerous. Thevesicles resolved spontaneously.Differential diagnosis includesherpetic lesions and transientneonatal pustular melanosis.

Figure 1.27. Lentigines are smooth,freckle-like, pigmented macules. Theyare usually present at birth, have ascattered distribution, and have beenconsidered by some to be a manifesta-tion of intrauterine erythema toxicumneonatorum. They usually disappearwithin 6 to 8 weeks.

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Figure 1.28. Another example oflentigines. Note the scattered distribu-tion of the lesions which were present atbirth. Lentigines should be different-iated from freckles (ephelides) which arered or light brown, well-circumscribedmacules usually less than 5 mm in dia-meter. Freckles are not seen in infancybut appear in childhood, especially onsun-exposed areas of the skin. In gen-eral, freckles are found in clusters.Because they normally do not appear inthe axilla, their presence in this area is astrong indication of neurofibromatosis.

Figure 1.29. Transient neonatal pustu-lar melanosis in an infant at the age of 3days. This is a benign self-limiting disor-der of unknown etiology characterizedby superficial sterile vesiculopustularlesions that rupture early. They presentas intact pustules as well as rupturedlesions which become evanescenthyperpigmented macules. These lesionsare usually present at birth and are seenin 0.5 to 2% of newborns. Over 90% ofthe infants with this condition areblack. The lesions are most often seen inclusters under the chin, and on the fore-head, neck, lower back, and the extrem-ities. They generally regress within 1 to2 months and the hyperpigmented areaeventually blends as the surroundingskin darkens.

Figure 1.30. Another example of tran-sient neonatal pustular melanosis inwhich the typical lesions are present.Note the vesiculopustular lesions andthe brown hyperpigmented macules.The lesions of transient neonatal pustu-lar melanosis are sterile on culture, andsmears of fluid from the vesicles demon-strate neutrophils and cellular debris.There are few or no eosinophils, in con-trast to the lesions of erythema toxicumneonatorum which reveal clusters ofeosinophils and a relative absence ofneutrophils. Differential diagnosis in-cludes erythema toxicum and staphylo-coccal, herpetic, or candidal infections.

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Figure 1.31. Sebaceous gland hyperplasia repre-sents a physiologic phenomenon of the newbornmanifested by multiple, yellow to flesh-coloredtiny greasy-looking papules that occur on the nose,cheeks, and upper lips of full term infants. Thesepapules, a manifestation of maternal androgenstimulation, represent a temporary disorder thatresolves spontaneously within the first few weeksof life.

Figure 1.32. A close-up view of sebaceous glandhyperplasia.

Figure 1.33. Milia occur com-monly on the face of 25 to 40% ofnewborn infants. Histologicallythey are small superficial inclusioncysts that result from retention ofkeratin and sebaceous materialwithin the pilosebaceous glands ofthe newborn, and appear as tiny 1-to 2-mm white or yellowish-whitepapules. The lesions are called “milia”because of their resemblance tomillet seeds. Milia are particularlyprominent on the cheeks, nose, chin,and nasolabial folds. The conditionis self-limiting and resolves withinthe first month of life. Persistentand numerous milia may be seenin association with other defects (e.g.,oral-facial-digital syndrome type I).

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Figure 1.34. Miliaria crystallina(sudamina) consists of clearsuperficial pinpoint vesicles with-out an inflammatory areola. Thelesions appear especially on thehead and chest. Each vesicle isrelated to a sweat gland, the ductof which has been obstructed oroccluded. The rash differs frommilia in that the vesicles lack thewhite opacity of milia, generallyappear a little later (in the 2ndweek), and are often related toexcessive warmth and humidity.The incidence is greatest in thefirst few weeks of life owing to therelative immaturity of the eccrineducts which favor poral closureand sweat retention.

Figure 1.35. Miliaria rubra(prickly heat) is characterized by small discrete erythematouspapules, vesicles, or papulovesicleswhich are surrounded by ery-thema. Lesions have a predilec-tion for covered parts of the bodywhere the baby gets overheated. Itis important to unwrap thesebabies and avoid excessive heat.

Figure 1.36. A close-up of thelesions of miliaria rubra. Notethe clear vesicles and erythemaof the surrounding skin.

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Figure 1.37. Infants with acne neonatorum have thetypical facial distribution of the comedones seen inacne in adolescence. The chest and back are rarelyinvolved. Neonatal acne appears to develop as a resultof maternal androgen stimulation of sebaceous glandsthat have not yet involuted to their childhood state ofimmaturity. Acne neonatorum is a common, transitory,self-limiting disorder and should not be mistaken for aninfection.

Figure 1.38. Intrauterine suckinglesions (sucking blisters) may present assmall intact or ruptured bullae and aremost commonly seen on the radialsurface of the wrist, dorsum of the hand,or dorsum of the fingers. If unruptured,as in this infant, they may be filled withsterile serous fluid or, if sucking is vigor-ous, there may be a hemorrhagic com-ponent. Intrauterine sucking lesions arean example of self-induced tissuedisruption in a normal newborn; thelesions are benign and require notherapy.

Figure 1.39. The intrauterine sucking lesions at thewrists in this infant have ruptured and left raw areaswhich will not heal as long as the infant continuessucking at the site.

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Figure 1.40. Another example ofan intrauterine sucking lesion onthe dorsum of the right hand.

Figure 1.41. The intrauterinesucking lesions may heal and pre-sent at birth with an area of scar-ring, as noted in this infant.

Figure 1.42. In upper panel, note another example of scarringat the left wrist in a normal newborn from an intrauterine suck-ing lesion. Below, this infant continued sucking its wrist afterbirth at the site of this lesion.

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Figure 1.43. Facial abrasions in this infant were self-inflicted. This type oflesion results from hyperactivity in an infant with long finger nails and isseen more commonly in postmature infants and in infants with drug with-drawal.

Figure 1.44. Abrasion of the nose (“sheet burns”) in aninfant with hyperactivity due to drug withdrawal. At the pre-sent time hyperactivity is most commonly seen with drugwithdrawal, but may occur in infants experiencing pain, con-genital hyperthyroidism, etc. The abrasions and erythemagenerally develop over prominent body parts such as the nose,ears, cheeks, elbows, and knees.

Figure 1.45. “Sheet burn” of the cheeks in a hyperactiveinfant who was lying in a pool of regurgitated gastric contents.A similar appearance could occur with hyperactivity in aninfant with drug withdrawal alone.

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Figure 1.46. Abrasions of theknees occurring in an infant withdrug withdrawal. This type oflesion, which occurs as a result ofrepeated hip flexion in a hyperac-tive infant, is very common.

Figure 1.47. This infant presentedat birth with an abraded area in theneck. The cord was around theneck three times and this wasthought to be the etiology of theabrasion.

Figure 1.48. The abrasions of the head and face in this normal infantoccurred with forceps delivery. There was rapid healing.

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Figure 1.49. Skin incisions overthe buttocks in an infant follow-ing cesarean birth.

Figure 1.50. Edema and ecchymoses of the face in a premature infant whowas delivered as a face presentation. These infants need to be checked foranemia and hyperbilirubinemia.

Figure 1.51. Suffusion of the face and head in an infant who had a tightnuchal cord. Note the difference in color of the face and head comparedwith the rest of the body.

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Figure 1.52. In this infant aged 6 days, note the healing abrasion from theapplication of forceps. In addition note the changes in the skin in thatthere is some reddish-purple discoloration and swelling with induration ofthe underlying subcutaneous tissue. This is an example of early subcuta-neous fat necrosis. Subcutaneous fat necrosis occurs in areas subjected toundue pressure such as by forceps.

Figure 1.53. Subcutaneous fat necrosis of the cheek inan infant following forceps delivery. In subcutaneousfat necrosis, which is usually detected towards the endof the first week of life, the lesions have an inflamma-tory or ecchymotic appearance. The underlying tissuemay be indurated and feels diffusely hardened. Withbreakdown of the subcutaneous tissue after several daysthere may be an area in the center which is fluctuant.If managed conservatively, spontaneous healing usu-ally occurs. This condition should not be mistakenlytreated as an infection.

Figure 1.54. This infant, aged 5 days and delivered as a breech presenta-tion, developed the extensive reddish-purple discoloration and swelling ofthe skin over the back with induration of the underlying subcutaneoustissue. This is an example of extensive subcutaneous fat necrosis. Withconservative treatment, improvement occurred within 2 to 3 weeks.Differential diagnosis includes sclerema neonatorum in which there isprogressive hardening of the subcutaneous tissue associated with severeillness of the infant. In sclerema the involved areas are hard and non-pit-ting and the palms and soles are spared.

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Figure 1.55. During delivery ofthis infant with a breech presenta-tion there was much manipulationand handling. He developedsevere generalized subcutaneousfat necrosis which presented asnumerous ecchymotic lesions withmarked induration of the skin.Any handling of this infantresulted in pain and discomfort.Over a period of several weeks thesubcutaneous fat necrosis im-proved but the infant developedhypercalcemia. His serum calciumlevel was 15.8 mg/dL. (There is acase report of an infant with sub-cutaneous fat necrosis who de-veloped a serum calcium level ofgreater than 20 mg/dL.)

Figure 1.56. This infant delivered by emergency cesarean birthwas an extremely difficult delivery and on the 4th day of life he developed an area of subcutaneous fat necrosis over each buttock as noted in the top panel. In the lower panel, note howpressure over these sites during delivery resulted in the subcuta-neous fat necrosis.

Figure 1.57. In this infant therewere several attempts at perform-ing a spinal tap for sepsis evalua-tion. Four days later he developedareas of subcutaneous fat necrosisover the lower lumbar area. Thesecould easily have been mistakenfor abscesses but were confirmedto be subcutaneous fat necrosisoccurring from the pressureapplied in performing the spinaltap. Note the mongolian spots.

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Figure 1.58. Calcification may occur in areas of subcuta-neous fat necrosis. In this radiograph note the soft tissuecalcifications occurring as a result of subcutaneous fatnecrosis.

Figure 1.60. This infant has a typical pig-mented skin dimple at the knee. The pres-ence of a skin dimple over a joint,pigmented or not, is normal. Skin dimplingis frequently noted in a relatively mild formwhere there has been prolonged intrauter-ine pressure upon the bony prominences,particularly at the elbows and knees.Normal skin dimples are most commonlynoted at the knee joints, over the lateralaspect of the elbows, over the acromionprocess, and in the lumbosacral area.

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DEVELOPMENTAL ABNORMALITIES OF THE SKIN

Figure 1.59. Calcifications occurred in thescapular area of this small premature infantas a result of subcutaneous fat necrosiswhich developed from vigorous chest phys-iotherapy.


Figure 1.61. A pigmented skindimple over the left shoulder.Normal skin dimples in generaltend to occur in areas where theskin is relatively tightly boundto the underlying bony prominences.

Figure 1.62. Nonpigmentedskin dimples in the iliosacralarea. These tend to be crease-shaped and may be multipleover the lower part of the back.If midline, they should be dis-tinguished from a pilonidalsinus.

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1.63Figure 1.63. Dimples in between thejoints over the long bones are consid-ered pathologic until proven otherwise.In this infant with congenitalhypophosphatasia, the skin dimple overthe middle of the tibia is a very typicalfinding. Abnormal (aberrant) skin dim-ples may occur at a location wherethere has been a closer than usual prox-imity between the skin and the under-lying bone structure during fetal life,resulting in deficient development ofsubcutaneous tissue at that locus. Suchdimples may be secondary either to aloss of subcutaneous tissue or to anaberrant bony promontory. These mayalso cause breakdown of the dermiswith ulcer formation.

Figure 1.64. An example of an abnormal skin dimple in aninfant with camptomelic dysplasia. Note the extreme pre-natal distortion of the bones with the development of theaberrant skin dimples over the abnormal bony prominences.The most common site for this type of abnormal skin dim-ple is over the apex of a severe curvature of the tibia in casesof fibular hypoplasia, but other areas can be similarlyaffected.

Figure 1.65. This infant withexstrophy of the cloaca sequencehad a dysplastic, markedly hypo-plastic right tibia with deformity ofthe right foot. Note the severity ofthe abnormal skin dimple.

Figure 1.66. Ulceration of theskin, which was present at birth inthis infant, occurred as a result ofintrauterine pressure on the fetus.

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Figure 1.67. The large skin foldon the back of this infant was hardand fibrotic. It was believed tohave occurred as a result of theskin being “pinched” in utero fromintrauterine constraint very earlyin gestation.

Figure 1.68. A midline occipitaldefect consisting of a tag with somecystic formation is seen in thisinfant. In any infant with a midlinelesion anywhere from the back ofthe neck to the lower end of thespine, it is mandatory to investi-gate the neural axis. The lesionsmay consist of small sinuses, cysts,or hemangiomas.

Figure 1.69. This infant pre-sented with a midline heman-gioma associated with a midlinetuft of hair over the spine. Furtherinvestigation confirmed the pres-ence of a neural axis defect. If hairtufts are associated with any ofthese lesions, the risk is evengreater.

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Figure 1.70. Pilonidal dimples areextremely common in neonates. Ifthe base of the dimple can be seeneasily, further investigation is notnecessary. If the dimple is very deepor appears to be a sinus tract it is important to do a neurologicevaluation.

Figure 1.71. Another example of a pilonidal dimple which is deep. Thisinfant’s MRI study was normal.

Figure 1.72. In this infant there isa midline defect over the distal endof the spine. With this type oflesion it is mandatory to do furtherstudies. MRI confirmed the pres-ence of a tethered cord syndrome.Note the “bandaid sign” in thisinfant confirming that the infanthad a spinal tap performed as partof a sepsis evaluation. This wascontraindicated in this infantbecause of the midline skin defect.

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Figure 1.73. A baby with atail. Vestigial tails are rarelyseen in the neonate. They mayconsist of soft tissue only, as inthis infant, or may containosseous structures.

Figure 1.74. Note the preauricu-lar and facial skin tags in this oth-erwise normal infant. Preauricularskin tags are extremely common,but the presence of skin tagsbetween the ear and corner of themouth would suggest a diagnosis ofGoldenhar’s syndrome.

Figure 1.75. Midline skin andtissue band between the jawand lower sternum in an other-wise normal infant.

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Figure 1.76. A midline skin tag of the chin. This consisted of softtissue only. The radiograph of the mandible was normal.

Figure 1.77. This infant has redundancy of the skin in the neckwhich was present at birth. It was related to the infant’s position inutero in that the head was flexed on the right upper chest, result-ing in the tight skin. There may be a small amount of redundantskin in the neck normally, but when there is enough to cause visi-ble folds or webs on the lateral neck, prenatal edema in the regionis almost certain to be the cause. Extensive redundancy of neckskin is seen in a number of dysmorphic conditions (e.g., Turner’sand Noonan’s syndromes). Once localized skin growth has takenplace, it does not easily reconstitute itself if the distending forcesare removed.

Figure 1.78. Mastitis neonatorum dueto physiologic breast engorgement is theresult of transplacental transfer of mater-nal estrogen to the fetus. Enlargement isgenerally symmetrical, as noted in thisinfant. Witch’s milk (which is chemi-cally identical to colostrum) may beexpressed from the breasts, but it is notadvisable to relieve the swelling byexpressing the milk since infection mayfollow.

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Figure 1.79. Another example of mastitis neona-torum which is asymmetrical in that it is moreprominent on the right than on the left. Mastitisneonatorum is noted more frequently in postmatureinfants. It subsides spontaneously over the course ofseveral weeks.

Figure 1.80. This infant has inclusion cysts ofthe right nipple. These require no treatment asthey resolve spontaneously.

Figure 1.81. Note that the nipples are in the5th intercostal space in this otherwise normalinfant. The nipple may be found anywherealong the milk line which extends from theaxilla to the pubis. Normally the nipple islocated at the 4th intercostal space.

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Figure 1.82. Notethe bilateral supernu-merary nipples in theanterior axilla andbilateral supernumer-ary nipples below thenormal nipples in thisblack infant.

Figure 1.83. Supernumerary nip-ples are especially common inmembers of darkly pigmentedracial groups. They occur any-where along the milk line and thesupernumerary breast tissue maypresent as an oval pigmented spotless than half the size of the nor-mal nipple, or may present asanother fully developed nipple.

Figure 1.84. In this infant there is a unilateral super-numerary breast on the left side. Supernumerarybreasts are rare. These are potentially functional and,like extra nipples, these structures occur along theembryonic milk line unilaterally or bilaterally, usuallybelow the normal site of breast placement. The nippleand areola are quite well developed, distinguishing thisanomaly from simple supernumerary nipples.

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Figure 1.85. Note that the nipples arewide-spaced and there is a right-sidedunilateral supernumerary nipple. Ingeneral, wide-spaced nipples occurrarely in infants with a normal chestconfiguration. Infants with Turner’s orNoonan’s syndrome have wide-spacednipples but have a shield-like chest.

Figure 1.86. This infant with Poland’sanomaly has absence of the nipple(athelia) on the right associated withthe absence of the pectoralis muscle.Athelia is rare but is seen unilaterallyin infants with Poland’s anomaly, andbilaterally in certain forms of ectoder-mal dysplasia. Hypoplastic nipples are usually poorlypigmented with a narrow or absent are-olar zone and little palpable breast tis-sue. Both sides are equally affected.Absence of the breast (amastia) is veryrare. There is no sign of nipple, areola,or mammary tissue.

Figure 1.87. Congenital scalp defect(aplasia cutis congenita) occurs mostcommonly as a single small defect ofthe scalp, 1 to 2 cm across, and is usu-ally located close to the normal site of aparietal hair whorl. The lesions have awell defined edge with a punched-outappearance, and may be multiple orinvolve a larger portion of the scalp.Etiology is unknown, and they heal withscarring leaving a zone of permanentalopecia.

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Figure 1.88. Scalp defects are afrequent finding in infants withtrisomy 13, as in this infant. Amore severe congenital scalpdefect may have a full thicknessabsence of the skin with largeareas of skin aplasia. This lesionmay occur as a result of in uterodisruption by an amniotic band.

Figure 1.89. In this otherwisenormal infant there is a large con-genital scalp defect associatedwith a bullous bleb.

Figure 1.90. A massive congeni-tal scalp defect which involvedthe skull and exposed the duramater.

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Figure 1.91. The severe congeni-tal scalp defect in this infantoccurred in the Adams-Oliver syn-drome, which is a disruptionsequence associated with limbreduction anomalies and scalp andskin defects as a result of amnioticbands.

Figure 1.92. The same infantshowing the marked aplasia cutiscongenita in the skin of theabdomen with dilated superficialcapillaries. This is an example ofaplasia of the skin affecting thetrunk as a result of amniotic bands.It is quite rare.

Figure 1.93. Congenital skindefect of the thigh in an infant. Itis not known whether this type ofdisruption results from an intrinsicabnormality of the skin itself orwhether aberrant bands of amni-otic tissue adhere to intact fetalskin. Some cases of scarring of theskin, especially if linear, have beenassociated with prenatal varicellainfections.

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Figure 1.94. Congenital alopeciais usually an autosomal recessivedisorder but may be an isolatedevent. Hair development begins atabout week 14 of gestation, andfrom gestational week 20 until birththe body is covered with fine lanugohair. This is gradually replaced dur-ing the first months of postnatal lifewith coarser, moderately pigmentedvellus hair. Alopecia or hypotri-chosis occurs in several syndromes(e.g., ectodermal dysplasia wherethe hair is sparse over the scalp,eyebrows, and eyelashes; progeria;Hallermann-Streiff syndrome; andcartilage-hair hypoplasia).

Figure 1.95. The scalp of thisinfant showed a patchy alopecia atbirth. A hair whorl is situated overthe part of the brain growing mostrapidly from about 16 to 19 weeksof gestation. The normal position isslightly lateral to the midline in theposterior parietal zone. It appearsthat this patch of alopecia is relatedto this. Physiologic “frictional”alopecia usually occurs over theoccipital region as a result of headrolling and friction producing hairloss on the back of the head. It ispoorly circumscribed and resolvescompletely once the infant is ableto change its position at will.

Figure 1.96. Hypertrichosis is seenin normal infants, especially in hispanic infants who tend to havemore hair than caucasian or blackinfants. In hispanics especiallythere may be hairy ears with longcoarse dark hair emerging from thelateral and posterior surface of thepinna.

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Figure 1.97. Hypertrichosis in aHispanic infant whose mother wasan epileptic treated with pheny-toin throughout her pregnancy.Hypertrichosis is associated withsyndromes such as Cornelia deLange’s, leprechaunism, etc. Ininfants with hypertrichosis associ-ated with syndromes, there may be alow anterior hairline which is espe-cially noted at the sides of the fore-head approaching the lateraleyebrows due to a widened “side-burn.”

Figure 1.98. Hypertrichosis in an infant with Cornelia de Lange’ssyndrome. Note also the synophrys, anteverted nostrils, and lack ofphiltrum. Synophrys (bridging of the eyebrows in the midline) is seenin Cornelia de Lange’s syndrome, Waardenburg’s syndrome, and inotherwise normal infants. The skin between the eyebrows usuallybears only fine vellus hairs, and when the brows encroach on this areathey produce the appearance of a single band of hair above the eyes.Straight eyelashes emerge from the lid margin at a steep angle andextend straight downward rather than exhibiting the gentle upwardcurve. Such eyelashes are seen in children with severe neuromusculardisease and may be caused by lack of normal muscle tone in the leva-tor palpebri superioris muscle.

Figure 1.99. This infant’s triangular nails fit intothe category of tapered nails that become progres-sively narrower and more hyperconvex as they growdistally. The nails generally reflect the size and shapeof the underlying distal phalanx (e.g., hypoplasticnail, narrow hyperconvex nail, short broad nail).

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Figure 1.100. In this infant withthe fetal hydantoin syndrome thenails are hypoplastic and distortedor absent over the short hypoplas-tic distal phalanges. Note the digi-talization of the thumbs. Total nailaplasia is very rare. At birth, someor all of the fingers bear a rudimen-tary nail bed. In nail-patella syn-drome, partial nail aplasia occurs,particularly in the thumbs andindex fingers.

Figure 1.101. Unusually broadnails of normal length are foundover digits with duplication of thedistal phalanx. They are also seenin Rubenstein-Taybi syndrome andLarsen’s syndrome.

Figure 1.102. Unusually broadtoes in another infant withRubenstein-Taybi syndrome.

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Figure 1.103. Petechiae are common in normal infants, particularly overthe back and buttocks. They usually disappear within a few days. Facialpetechiae are commonly seen in infants in whom there was a nuchal cordor where there was abnormal delay following delivery of the head and neckbefore the trunk and shoulders were delivered.

Figure 1.104. The petechiae in thisinfant were associated with severebirth asphyxia. The platelet countwas 90,000/mm3 and the petechiaeresolved spontaneously within a fewdays.

Figure 1.105. Macular hemangioma(“angel’s kiss”) of the eyelids in a newborninfant. Vascular nevi occur in up to 40% ofnewborns. They are divided into three groups(salmon patches, hemangiomas, and vascularmalformations) and are classified by their his-tology and the degree of involvement of theskin and subcutaneous tissues. The most com-mon are the salmon patches (macular heman-gioma, nevus flammeus). They consist ofdilated capillaries in the superficial layers ofthe dermis and are noted most commonly onthe face, the midforehead, the glabella,philtrum, and the nape of the neck. Multiplesites are often affected.

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VASCULAR DISORDERS AND MALFORMATIONS

Figure 1.106. Macular hemangioma of the eyelids, glabella, and face.Macular hemangiomas (telangiectatic nevi) are poorly defined, bright redin color, and blanch easily with pressure, but the blood returns rapidly. Ingeneral these capillary hemangiomas become less visible in the first year oflife as the skin becomes less translucent.

Figure 1.107. A macularhemangioma (salmon patch) inthe nape of the neck is com-monly called the “stork-bite”nevus. These are the most com-mon of the macular heman-giomas and fade gradually, butare more likely to persist thanother macular hemangiomas.

Figure 1.108. An infant at age5 months with a “stork-bite”nevus.

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Figure 1.109. A macularhemangioma of the left knee inan infant. Lesions on the trunkand limbs may be extensive.

Figure 1.110. A nevus anemi-cus present at birth on the backof an infant. Note that thenevus is pale and has promi-nent capillaries. It may be theprecursor of a strawberry nevusin that it starts as a fine, thread-like telangiectasis surroundedby an area of localized pallor.Both strawberry nevus andnevus anemicus involute spon-taneously.

Figure 1.111. Another example of anevus anemicus present at birth on theleft thigh. Capillary hemangiomas(strawberry nevi) may be present atbirth, but generally develop during thefirst few postnatal weeks as pale orslightly reddened, well-demarcated zonesof skin a few millimeters to several cen-timeters in diameter. They may occur onany area of the body but are seen mostcommonly on the head and neck (40%)and the trunk (30%). During the firstmonths of life, rapid growth occurs andthe lesions become elevated above thesurrounding skin with a spongy consis-tency, and attain a bright red to purplecolor. Although compressible, theyblanch with gentle pressure but seldomempty completely.

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Figure 1.112. A capillary hemangioma (straw-berry nevus) which developed in the upper tho-racic region over the course of several weeksafter birth is noted in this infant at the age of 3weeks. The irregular surface with sharp demar-cation is typical of a “strawberry” hemangiomawhich consists of dilated new capillaries in thedermal or subdermal area. The classic straw-berry hemangioma is a raised, bright- or pur-plish-red lobulated tumor with well-definedborders and minute capillaries protruding fromits surface, hence its “strawberry-like” appear-ance. Their history is usually one of continuedrapid enlargement during the first few monthsof life followed by gradual spontaneous regres-sion which occurs by central involution with-out scarring. In 50% of cases they resolve by theage of 5 years, in 70% by the age of 7 years, andin 90% by the age of 9 years.

Figure 1.113. In this twoand a half months old infantwith a birthweight of 585 g,note the small strawberrynevus (figure left). This vas-cular tumor is apparentlycaused by a local persistenceof angioblastic cells that giverise to a plexus of thin-walled capillaries with poorvenous drainage. This con-tinued to enlarge and at theage of three and one halfmonths, note the markedenlargement of the nevus inthe underlying tissue (figureright).

Figure 1.114. This 8-week-old premature infant with a birthweight of 900 gdeveloped several strawberry nevi on the scalp at the age of 6 weeks. Note therapid growth over a 2 week period. Hemangiomas are rarely seen in prema-ture babies with a gestational age of less than 34 weeks. As the infantbecomes older, hemangiomas may develop.

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Figure 1.115. This infant shown in Figure1.114, now at the age of 3 months, developed acystic swelling over the scalp in the first few weeksof life. The cystic swelling increased gradually insize; note the bluish hue. On removal this wasconfirmed to be a strawberry hemangioma. Notethat strawberry hemangiomas may have a largecomponent visible on the surface of the skin, ormay be covered by the skin, thus obscuring theircharacteristic appearance.

Figure 1.117. The capillary hemangioma involving the rightside of the face in this infant demonstrates the fact thathemangiomas may expand sufficiently to interfere with func-tion or may evidence bleeding or superficial infection. In thisinfant there would be marked interference with developmentof normal vision and, if untreated, this would lead to astigma-tism and other problems. In such instances, treatment withsteroids or laser surgery may be indicated.

Figure 1.116. In some infants the lesion mayappear to be a hemangioma but, as in this exam-ple, on biopsy the diagnosis was a hemangioen-dothelioma of the right chest.

Figure 1.119. Another example of Sturge-Weber syndrome in which the distributioninvolves both the 1st and 2nd branch of thetrigeminal nerve. Note the glaucoma of the righteye. In Sturge-Weber syndrome, lesions stop atthe midline. When extensive facial involvementis present, there may be an associated glaucoma(buphthalmos). As this is one of the neurocuta-neous syndromes, a CT scan of the head shouldbe done to exclude intracranial involvement. Radio-graphs of the skull may reveal unilateral curvilinear,double-contoured lines of calcification in thecerebral cortex (“railroad track calcification”).The intracerebral vascular abnormalities lead tobrain atrophy and ocular lesions (optic atrophy).

Figure 1.120. This infant with involvementof the trunk and limbs is another example ofSturge-Weber syndrome. There were bilateralcongenital glaucoma, seizures at the age of 6days, and an abnormal CT scan. In Sturge-Weber syndrome, seizures occur in 80% ofinfants, mental retardation in a high percent-age, and an associated glaucoma in 40 to 50%of the infants, especially if both the oph-thalmic and maxillary branches of the trigemi-nal nerve are involved.

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Figure 1.118. Port wine stain (nevus flammeus)is another macular hemangioma which representsa regional dilatation and enlargement of maturecapillaries. It usually affects the skin of the faceand neck, and the lesions are sharp-bordered andrange in color from pale purple to deep burgundy.It usually does not indicate underlying abnormal-ities unless it extends into the area of distributionof the ophthalmic branch of the trigeminal nervethat serves the facial skin over the eyelid and upinto the brow where, as in this infant, it maysignal the presence of the intracranial vascularanomalies of Sturge-Weber syndrome (enceph-alotrigeminal angiomatosis).


Figure 1.121. Note the widespreadinvolvement of the trunk and limbs in thesame infant. The likelihood of Sturge-Weber syndrome is greater when nevi suchas these are present on the trunk and thelimbs.

Figure 1.122. Phacomatosis pigmentovas-cularis is a rare condition in which there isa port wine appearance (as in Sturge-Webersyndrome) in addition to patches of slate-gray hyperpigmentation which clinicallyresemble mongolian spots. The infant alsohad a glaucoma of the left eye. This condi-tion has been associated with glaucoma,seizures, and skeletal abnormalities.

Figure 1.123. A large cavernous heman-gioma was present on the scalp of this infantat birth. These are raised lesions and oftendo not appear until the infant is a few weeksof age. Deep cavernous hemangiomas arebased on the localized failure of normalangiogenesis. They grow slowly after birthand have a blue color because of their sitebelow the dermis. There is a soft movablenonpulsatile mass which feels like a “bag ofworms.” Blood flow through these tumors isvery slow and, therefore, they do not com-press easily or blanch with pressure.

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Figure 1.124. Cavernous hemangiomas occurmainly on the head and neck but may presentanywhere such as this large cavernous heman-gioma of the right knee. Thrombocytopenia dueto platelet trapping, and marked vascular shunt-ing leading to high output congestive heart fail-ure may be complications associated with largecavernous hemangiomas.

Figure 1.125. This infant with Kasabach-Merrittsyndrome (cavernous hemangioma with throm-bocytopenia) had a large cavernous hemangiomaof the right lower thigh and knee and a plateletcount of 54,000/mm3. These infants may developdisseminated intravascular coagulopathy becauseof the consumption of the coagulation factors.

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Figure 1.126. Thislarge cavernous hem-angioma of the left sideof the chest wasasymptomatic and theinfant had a normalplatelet count.


Figure 1.127. The massive cavernoushemangioma involving the left leg andfoot of this infant was compromisedduring in utero life, resulting in gan-grene. The hemangioma was removedsurgically after birth. In large cavernoushemangiomas the underlying structuresmay be involved, such as in this infantwhere the underlying osseous structureswere involved.

Figure 1.128. The massive cavernoushemangioma of the right upper extrem-ity in this infant resulted in high outputcongestive heart failure in utero. Therewas marked anasarca with a total pro-tein of 2.8 mg/dL, hematocrit of 20%,and platelet count of 26,000/mm3.

Figure 1.129. A large capillaryhemangioma (nevus flammeus) involvingthe right shoulder and right upperextremity resulting in an asymmetrichypertrophy of the shoulder and rightupper extremity. This constitutes theKlippel-Trenaunay syndrome. Unilateraldistribution predominates and theremay be disproportionate growth. Hyper-trophy is not always present at the siteof the vascular malformation. The causeof the hemihypertrophy is not definitelyknown, but it is suggested that it maybe due to a local increased blood supplyto the area.

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Figure 1.130. The large cavernoushemangioma involving the neck,chest, and axilla of this infant wasassociated with numerous othersmall hemangiomas over the bodyand resulted in hemihypertrophy ofthe right upper arm, the right leg,and the left foot. This is anotherexample of Klippel-Trenaunay syn-drome. In Klippel-Trenaunay syn-drome one should always check forhemangiomata of the viscera, brain,and eyes in addition to the involve-ment of the skin.

Figure 1.131. The lower extremi-ties of the same infant as in Figure1.130 with Klippel-Trenaunay syn-drome shows the hypertrophy of theright leg and the left foot.

Figure 1.132. Multiple hemangiomatosis (dif-fuse neonatal hemangiomatosis) may presentsolely with cutaneous involvement, as noted inthis infant, or with systemic involvement (ofthe liver, brain, etc.). There are numerouswidely disseminated, small, red to dark bluepapular cutaneous hemangiomas which areusually present at birth or develop within thefirst few weeks of life. In infants with cutaneousinvolvement only, prognosis is good. In infantswith systemic involvement, prognosis is variable.

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Figure 1.133. A close-up view of themultiple hemangiomata in the sameinfant as in Figure 1.132.

Figure 1.135. In cutis marmoratatelangiectatica congenita (congenitalgeneralized lymphangiectasia), dilatedsuperficial venous and capillary chan-nels are usually noted at birth. Theskin appears as a reticulated networkwith white insulae in between. Theclassic appearance of reddish-bluereticulation of the skin changes withcrying (becomes increasingly red) orother stimulation (becomes livid withcooling). Most cases are generalized,such as in this infant, but there maybe segmental or localized involvement.

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Figure 1.134. Multiple heman-giomatosis in an infant with systemicinvolvement. There were multiplehemangiomata in the liver and sev-eral hemangiomata in the gastroin-testinal tract and brain in this infant.At the age of ten days the infantdeveloped hematemesis and abdomi-nal distention. At surgery it was notedthe infant had massive hemorrhagefrom a hemangioma in the duode-num. If systemic involvement is sus-pected, liver and spleen scans orhepatic angiography may confirm thediagnosis. (See Figure 2.147 ofVolume V for an example of systemicinvolvement in multiple heman-giomatosis.)

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Figure 1.136. Another infant with cutis marmoratatelangiectatica congenita. The condition may extendfor the first few weeks and usually persists throughoutlife, but eventually improves during childhood. Rarely,small areas of superficial ulceration may develop.

Figure 1.137. Cutis marmoratatelangiectatica congenita inwhich the distribution is segmen-tal. The etiology is unknown,but it seems to represent adevelopmental ectasia of bothcapillaries and veins.

Figure 1.138. Lymphedema in an infant with Turner’ssyndrome shows pitting edema on the dorsum of thehand. The diffuse soft tissue swelling in lymphedema iscaused by increased accumulation of lymph due toinadequate lymphatic drainage. Lymphedema may beprimary (congenital) or secondary.

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Figure 1.139. Lymphedema of both feet in an infantwith Turner’s syndrome. The area is swollen and firm atbirth and is characterized by pitting on pressure.

Figure 1.140. Milroy’s disease is an inherited autoso-mal dominant condition which presents with the typi-cal bilateral lymphedema in the lower extremities. Thecondition occurs as a result of absence of lymphaticsand is always confined to the legs and feet.

Figure 1.141. Lymphangioma simplex of the anteriorchest present at birth in an infant. There are fourmajor forms of lymphangiomas: lymphangioma sim-plex, lymphangioma circumscriptum, cavernous lym-phangioma, and cystic hygroma. Lymphangiomasimplex is a solitary, well-circumscribed, flesh-coloreddermal or subcutaneous tumor. It may occur anywhereon the subcutaneous or mucosal surface and is seenmost commonly on the neck, upper trunk, proximalextremities and tongue. The surface is generallysmooth and it may remain stable or grow quickly.

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Figure 1.142. On removal, thismass attached by a stalk to theright side of this infant’s face wasconfirmed to be a typical simplelymphangioma. The small preau-ricular tag was not associated withany other pathology.

Figure 1.143. Circumscribed lym-phangioma of the left lower extrem-ity in a neonate. Lymphangiomacircumscriptum is the most com-mon form of lymphangioma. It ischaracterized by groups of deep-seated, thick-walled vesicles thathave the appearance of “frogspawn” or “grape clusters.” Thecommon sites of involvement arethe proximal limbs, shoulders,neck, axilla and adjacent chestwall, perineum, inguinal folds,tongue, and mucous membranes.

Figure 1.144. A large cavernouslymphangioma of the left axilla andanterior chest present at birth in aninfant. Cavernous lymphangiomaconsists of diffuse soft tissue masses oflarge cystic dilatations of lymphaticvessels in the dermis and subcuta-neous tissue, and may involve theintermuscular septa. The lesionsare ill-defined and frequently in-volve large areas of the face, trunk,and extremities. They may occur inthe tongue, resulting in macroglossia.Lymphangiomas frequently have ahemangiomatous component(hemangiolymphoma) so that someof the vesicles are filled with fresh oraltered blood. Treatment is surgicalbut recurrences are common.

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Figure 1.145. A large cavernous lymphangiomaaffecting the right gluteal region and proximallower extremity of an infant. There was noosseous involvement.

Figure 1.146. Cystic hygroma of the right side ofthe neck with involvement of the mucous mem-branes. Cystic hygroma is a benign loculated cys-tic mass which is soft, diffuse, impressible, andtranslucent due to accumulation of fluid in thelymphatics. The commonest sites are in the neck(hygroma colli), axilla, and upper arm. Rarelythey are seen in the groin or popliteal fossa. Theydo not resolve spontaneously. Surgical treatmentmay be complicated and recurrences are uncom-mon following complete removal.

Figure 1.147. Transillumination of the cystic hygroma inthe same infant as in Figure 1.146. Note the extent ofinvolvement of the neck and mouth.

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Figure 1.148. This infant witha massive diffuse lymphangioma(elephantiasis congenita angi-omatosis or elephantiasis lym-phangiectatica) had severelycompromised respiration fromthe tumor and required trach-eostomy. Note the large area ofnevus present superficially overthe anterior part of the chest.Biopsy diagnosis was a hemangi-olymphoma. Note the redness ofthe area which was related tosecondary infection, a commoncomplication.

Figure 1.149. In another infantwith elephantiasis congenitaangiomatosis, note the massiveinvolvement of the right lowerextremity. Diffuse angiomas (ele-phantiasis lymphangiectatica) arelarge, ill-defined cystic dilatationsinvolving the skin, subcutaneoustissue, muscles, and mucous mem-brane. They may involve thetrunk, extremities, and large areasof the face, lips, or tongue. Thereis marked enlargement of theaffected areas as a result of inva-sion by the cystic lymphatics. Theareas may have a red or purplishcolor because of the presence in thevesicles of blood mixed with lymph.

Figure 1.150. There may be iatrogenic causes ofdelayed pigmentation, such as a hypopigmentedarea over the skin where chest electrodes or tapehave been placed. These hypopigmented areasoccurring as a result of irritation or breakdown ofthe skin darken with increasing age and blend withthe surrounding skin.

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PIGMENTATION ABNORMALITIESVitiligo (leukoderma) is a condition which is not commonly seen in the neonatal period. The irregularly outlinedhypopigmented areas seen in vitiligo often have hyperpigmented borders, and the patches may enlarge over aperiod of several years but will not darken over time. When areas of hypopigmentation are noted they must bedifferentiated from the ash leaf appearance of tuberous sclerosis.


Figure 1.151. Ash leaf spots arefound on the skin of the trunk,buttocks, and limbs in childrenwith tuberous sclerosis. These dull,white areas may be linear or oval,measuring 1 cm across or less.They tend to be sharply pointed atone end and rounded at the other.Café-au-lait spots may be present.Ash leaf spots lack the characteris-tic milky-white appearance oflesions of vitiligo becausemelanocytes are present in fairnumbers but the melanosomes arepoorly pigmented.

Figure 1.152. Examination ofthe lesions under the Wood’s filtershows the typical ash leaf spots inthe same infant with tuberoussclerosis. Tuberous sclerosis is aneurocutaneous syndrome which,in addition to the cutaneouschanges, has systemic manifesta-tions in 80 to 90% of cases. Theseinclude central nervous systeminvolvement (seizures, mentalretardation), cardiac tumors(rhabdomyomas), renal hamar-tomas, retinal lesions, and osseouschanges. CT scan may showtumor-like nodes in the cerebralcortex.

Figure 1.153. A close-up of a typical ash leaf macule in tuberous sclerosisshowing the characteristic hypopigmented leaf shape. In tuberous sclerosis,shagreen patches are also noted; these are zones of slightly thickened andfirm, yellow, irregularly outlined skin, a few centimeters across, with a finelydimpled surface resembling the skin of an orange. They are usually foundon the flanks and back.

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Figure 1.154. This is a black infant with totalalbinism, an autosomal recessive condition in whichtotal lack of pigment is characteristic. Albinism is anuncommon disorder of melanin synthesis manifestedby a total lack of pigmentation of the skin, hair, andeyes. It occurs in two forms: oculocutaneous and ocu-lar. In oculocutaneous albinism there is generally adecrease or absence of pigment in the eyes, skin, andhair; in ocular albinism only the eye pigment is consis-tently abnormal. If the iris does not have the typicalpink appearance seen in albinism but has a bluish-greypigmentation, it is less likely that the child will bephotophobic.

Figure 1.155. Another infant with albinism with her mother. Note thestriking difference in pigmentation of mother and baby. Albinism ischaracterized by varying degrees of reduction of pigment in the skin andhair, translucent irides, hypopigmented ocular fundi and an associatednystagmus. Affected children have been called “moon children”because they have marked photosensitivity and photophobia, and pre-fer to go outdoors only at night.

Figure 1.156. Close-up of the same infant as in Figure 1.154 showingthe total lack of pigmentation.

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Figure 1.157. Mother and infant with tyrosinase-positive oculocutaneousalbinism. Melanocytes or melanosomes are present in the affected skin andhair in normal numbers but, although they are tyrosine positive, fail to pro-duce normal amounts of melanin in the areas of leukoderma or poliosis.There are many variants of oculocutaneous albinism but tyrosinase-posi-tive or tyrosinase-negative are the most common. These are so designatedon the basis of pigment production in plucked hair incubated in tyrosine.

Figure 1.158. In partial albinism(poliosis circumscripta; piebaldism)there are hypopigmented areas ofthe scalp. Depigmented areas mayalso occur on the torso or theextremities with the exception ofthe back, hands, and feet. Decreasedhair pigmentation (poliosis) is mostoften seen close to the anterior hair-line either centrally or to one side of the midline. This condition(piebaldism) is seen in normal indi-viduals and may follow an autoso-mal dominant inheritance.

Figure 1.159. In Waardenburg’s syndrome, an autosomal dominant con-dition, a white forelock is characteristic. It is a form of partial albinism(poliosis). In addition to the white forelock, there is dystopia canthorum(lateral displacement of the medial canthi and lacrimal puncta of the lowereyelids), synophrys, heterochromia iridis, broad nasal root, and congenitaldeafness. If the inner canthal distance divided by the interpupillary dis-tance is greater than 0.6, this lateral displacement of the inner canthi mayhelp confirm the diagnosis.

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Figure 1.160. Another example ofWaardenburg’s syndrome. Note the whiteforelock and the dystopia canthorum.

Figure 1.161. Café-au-lait spots, presentin this twin infant with neurofibromatosis,are skin lesions caused by hyperpigmenta-tion of the basal epidermal cells. They maybe seen in healthy children, as well asthose with neurofibromatosis, tuberoussclerosis, and the Russell-Silver syndrome.They range in size from a few millimetersto several centimeters in diameter andhave a color slightly darker than that ofthe surrounding skin. The most commontype have edges that are fairly smooth andquite clearly demarcated (“coast of Cali-fornia”). A second type of café-au-lait spothas a much more jagged, irregular border(“coast of Maine”) and is usually larger andsolitary. Such lesions are seen in McCune-Albright polyostotic dysplasia.

Figure 1.162. In this infant with neurofi-bromatosis note the multiple café-au-laitspots. About 5% of white infants andalmost 15% of black infants have one suchspot. Café-au-lait spots are somewhatdarker in color in black infants than incaucasian infants. Neurofibromatosis is anautosomal dominant condition in whichthe presence of 5 or more café-au-lait spotsgreater than 0.5 cm in diameter in younginfants is diagnostic of the disorder.

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Figure 1.163. Another infant with neurofibro-matosis. The presence of a single café-au-lait spotin the axilla may be diagnostic of this disorder.Crowe’s sign (axillary freckling) appears as multi-ple 1- to 4-mm café-au-lait spots in the axillaryvault and is seen in 25 to 50% of patients withneurofibromatosis.

Figure 1.165. A close-up ofthe face of the same infant as inFigure 1.164 with familial pro-gressive hyperpigmentation.Histopathologically the mostdistinctive manifestation con-sists of heavy melanization ofthe basal cell layers.

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Figure 1.164. Familial progressive hyperpigmen-tation in a neonate is a benign form of familialhyperpigmentation that has been reported only inblack families. The mother was affected and,including this infant, she had five affected chil-dren. This dominantly inherited condition pre-sents at birth as irregular patches and streaks ofhyperpigmentation which increase in size, num-ber, and confluence with age. The pigmentationlater appears in the conjunctivae and buccalmucosa, and extensive areas of the skin andmucous membranes are involved.

Figure 1.166. Note the marked hyperpigmentation in theskin of the mother of the infant shown in Fig. 1.165. Alsonote the pigmentation in the conjunctivae.

Figure 1.167. A pigmented nevus(junctional nevus) is a flatmelanocytic nevus. The lesions aresuperficial, flat, discrete, brown, andhyperpigmented. There are usually afew lesions and they have a sharplydemarcated border. Their color mayvary from brown to black and theyare caused by excessive numbers ofmelanocytes (nevocytes) at the der-mal-epidermal junction. In someinstances, proliferation of nevocytesdown into the dermis occurs, givingrise to a raised, more or less darklypigmented papular or verrucouslesion. These nevi are usually benignand the potential for malignantchange is minimal but is greater forlesions that appear after birth.

Figure 1.168. Another example of apigmented nevus. Three types of pig-mented nevi are identified: intrader-mal, junctional, and compound.Intradermal nevi lie within the der-mis and occur most frequently inadults. Halo nevus is the term givento a darkly pigmented junctionalnevus that is surrounded by a palezone of depigmented skin. This halorepresents the body’s immuneresponse to the nevocytic cells,which is affecting melanocytes inthe surrounding normal skin for ashort distance.

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Figure 1.169. The pigmented nevusof the tongue is another example of a junctional nevus in an unusuallocation.

Figure 1.170. A pigmented nevus onthe scalp obviously has associated hairand, because of this, tends to be muchcoarser in texture. These junctionalnevi of the scalp may cover large areas.

Figure 1.171. In this infant there is a large benign hairy pigmented nevus.These nevi may be pale at first with fine vellus hairs, but increasing pig-mentation takes place during the first year of life, often with the growth ofdark coarse hair. The hyperpigmented zones may be extremely large withnumerous satellite lesions. They may occur anywhere on the body and arevariable in size. Approximately 10% of giant hairy nevi develop into malig-nant melanomas.

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Figure 1.172. A congenital giant pigmented nevus isextensive and sharply outlined. The hyperpigmentedzones may be extremely large, covering the trunk, espe-cially the lower trunk (“garment” nevus or “bathingtrunk” nevus). “Garment” nevi involve a very largebody surface area (e.g., the entire back or an extremity).The surface of these lesions is nodular or raised withfleshy elements and has a somewhat leathery texture. Itmay have dark, coarse hairs within the lesion.Histologically this would be classified as a compoundnevus. A junctional nevus lies only on the epidermalsurface and is flat, smooth, and relatively small.Junctional nevi over time may become compoundnevi. Compound nevi possess the features of bothjunctional and intradermal nevi in that nevus cells areseen within the dermis as well as within the epidermis.They tend to be more elevated and vary from a slightlyraised plaque to a lesion of a somewhat more papillo-matous nature.

Figure 1.173. A large “gar-ment” nevus involving most ofthe trunk and perineum. Ininfants with a giant “garment”nevus there may be widelydisseminated pigmentedpatches which represent sat-ellite melanocytic nevi. Inthese infants there is a propen-sity for malignant change, andtherefore an aggressive surgicalapproach is justified.

Figure 1.174. This infant with a large “garment”(“bathing trunk”) nevus had skin breakdown withulceration in utero and presented at birth with thisappearance. Examination of the placenta showednumerous secondary pigmented nevi in the placentaltissue. Histopathologic examination of the nevi in theplacenta showed numerous melanoma cells. Themother was normal.

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Figure 1.175. A large pigmented nevusover the midline in the thoracolumbar areaof this infant may be significant in that anylesion located in the midline over the spineshould alert one to the possibility of aneural tube defect. The faun-tail nevus is anelongated patch of dark skin bearing abun-dant long dark hair located in the midlineover the lumbar spine or sacrum. It is a rarebut helpful indicator of a tethered spinal cord.

Figure 1.176. The multiple pig-mented nevi involving the scalp andtrunk in this infant are characteristicof the neurocutaneous melanosissequence. There are dark pigmentedmultiple nevi, which are sometimeshairy and are more extensive in abathing trunk distribution over thelower trunk, abdomen, and lowerthighs. There may be leptomeningealinvolvement with nests and sheets ofmelanoblasts, most striking at thebase of the brain. This may lead tohydrocephalus, seizures, and deterio-ration of central nervous systemfunction. In these cases, cells con-taining melanin may be detected inthe cerebrospinal fluid.

Figure 1.177. A close-up of thelesions in the lumbosacral andgluteal areas in the same infant asin Figure 1.176.

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Figure 1.178. Another infantwith the neurocutaneous mel-anosis sequence. The finding ofa single giant nevus with nu-merous satellite lesions is notuncommon in this disorder.Cutaneous melanosis is evidentat birth and central nervoussystem function may be normalinitially, but seizures and men-tal deterioration occur later.The risk of malignant mela-noma degeneration is 10 to 15%.

Figure 1.179. Mastocytosis (urticariapigmentosa) is a mast cell infiltrativedisorder which presents at birth ordevelops during the first few weeks oflife. The lesions may be solitary or ingroups, as in this infant. Macules,papules, nodular lesions, vesicles, andbullae may all be signs of mastocyto-sis. They vary in color from a slightlyreddish appearance to a tan or brownappearance. Spontaneous remissionof the skin lesions usually occurs.Skin biopsy shows mast cell infiltra-tion.

Figure 1.180. In about 5% of cases,mastocytosis is localized. In the figureon the left there is a solitary tan tobrownish-colored, slightly raisedlesion on the back. As seen in the fig-ure on the right, rubbing the skin overthis area results in dermatographism(Darier’s sign) due to histaminerelease. Nodular forms of mastocytosismust be differentiated from xanthomaand juvenile xanthomagranulomas.

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Figure 1.181. This is a lesion of bullous mastocytosis(urticaria pigmentosa) that presented at birth in thisinfant as a solitary bullous lesion on the sole of theright foot. Note the area of redness surrounding thelesion (dermatographism) following examination ofthe lesion. Spontaneous remission of the skin lesionsusually occurs. It is rare for lesions to appear after theage of 3 years. Differential diagnosis includes bullousimpetigo, epidermolysis bullosa, incontinentia pig-menti, and bullous congenital ichthyosiformerythroderma.

Figure 1.182. In this infant witha systemic skin rash present atbirth, the diagnosis of urticariapigmentosa was confirmed. In sys-temic mastocytosis there ishepatosplenomegaly and boneinvolvement. Biopsy shows mastcell infiltration. In localized mas-tocytosis, which occurs in about5% of all cases of mastocytosis,spontaneous remission of the skinlesions usually occurs. Prognosis isusually good, provided that thereare no signs of systemic mastocy-tosis (hepatosplenomegaly, boneinvolvement).

Figure 1.183. Incontinentia pigmenti (Bloch-Sulzberger syndrome) is considered to be a neurocuta-neous syndrome seen only in female infants (X-linkeddominant, generally lethal prenatally to the male). Ithas four cutaneous phases. In this infant note the earlymaculopapular appearance without vesicles or pigmen-tation. The lesions contain eosinophils, and bloodeosinophilia is frequently present. In the next phasethere are many vesicular lesions. These then becomebullous and pustular. The bullae may be superseded byverrucous lesions in the same distribution, and afterthese disappear, whorls of hyperpigmentation appear.In the final phase, hypopigmented patches may occur.The patterning of the skin abnormalities follows thepath of Blaschko’s lines, which represent the course ofearly migration of primordial skin cells progressingfrom the dorsal to the ventral midline.

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Figure 1.184. Within the firstmonth of life in incontinentiapigmenti, the vesicular phasedevelops as noted in this infant.Note the linearly arranged vesi-cles and red nodules on theflexor surface of the upper andlower extremities. The blistersmay cluster in a bizarre arrange-ment if they are numerous.

Figure 1.186. In this phase of incontinentia pigmenti, vesiclesand bullae have formed and these have broken down with someincreased pigmentation.

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Figure 1.185. Another infant with incontinentiapigmenti. Note the maculovesicular pigmentedappearance of the skin in a swirling pattern. The infantdeveloped seizures on the 2nd day of life. Systemicmanifestations occur in about 70% of infants (20% atbirth). These include central nervous system (micro-cephaly, seizures, and retardation), ocular (cataracts,retinal dysplasia, etc.), osseous (hemivertebrae, extraribs, hemiatrophy, etc.), dental (hypodontia, etc.), andoccasionally cardiac abnormalities.

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Figure 1.187. A close-up of theright upper extremity showing thevesicles with some verrucous ele-ment and increasing pigmenta-tion with a tendency to followBlaschko’s lines.

Figure 1.188. In a later phase ofincontinentia pigmenti, between 6 to12 months of age, red-brown, hyper-pigmented lesions appear in a sym-metric distribution on the arms andlegs while other lesions haveimproved. Note the bizarre distribu-tion of the lesions and the pigmenta-tion. The pigmented zones have veryirregular patterns and tend to followthe path of Blaschko’s lines. Theyhave a roughly V-shaped configura-tion over the back, a wavy S-shapeddistribution over the anterior trunk,and a longitudinal orientation overthe limbs. These lesions tend to persist.

Figure 1.189. A close-up of theright lower extremity of an infantwith incontinentia pigmenti.Note the presence of vesicles andthe typical pattern of swirledhyperpigmentation of the skinthat has been compared to that ofa marble cake. This is presentmainly on the extremities andtrunk, and increases in intensityuntil the 2nd year of life. It maypersist many years, then graduallyfades.

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Figure 1.190. A close-up of thelower trunk, buttocks, and extremi-ties showing the pigmentarychanges which tend to follow thepath of Blaschko’s lines.

Figure 1.191. In incontinentiapigmenti achromians (hypome-lanosis of Ito), a neurocutaneoussyndrome, the distribution ofhypopigmentation is similar to thatof the hyperpigmented areas seen inincontinentia pigmenti. Note thatthere is a pattern of swirled hypo-pigmentation which looks like aphotographic negative of the hyper-pigmented streaks seen in inconti-nentia pigmenti. This disorder isassociated with seizures and mentalretardation. The whorls may appearwithout the prior development ofvesicles or bullae.

Figure 1.192. The back of thesame infant as in Figure 1.191 show-ing the distribution of the hypopig-mented lesions, again demonstratingthese bizarre patterns which look likea photographic negative of thehyperpigmented streaks seen inincontinentia pigmenti and whichtend to follow Blaschko’s lines. In afew patients, the lesions may bepatchy and confined to relativelylimited areas of the body. In mostcases, however, the hypopigmentedareas are extensive, often bilateral,and appear to be more pronouncedon the ventral surface of the trunkand the flexor surface of the limbs.

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Figure 1.193. Epidermal nevus on the scalppresent at birth. These are raised verrucouslesions of variable size, sometimes round butmore commonly linear, and tend to followBlaschko’s lines. They may appear later ininfancy and are slightly hyperpigmented or yel-lowish in light-skinned infants, while appearingdark brown to black in darker skinned infants.An epidermal nevus must be differentiatedfrom a nevus sebaceus of Jadassohn.

Figure 1.194. Another example of an epider-mal nevus on the scalp which was present atbirth but had a more verrucous and hyperpig-mented appearance. An epidermal nevus is abenign congenital disorder that is character-ized by circumscribed hyperkeratosis andhypertrophy of the epidermis. The diagnosiswas confirmed by skin biopsy.

Figure 1.195. A linear epidermal nevus of theback of the left thigh and leg which was pre-sent at birth. In this infant note the linearityand the increased pigmentation of the lesionson the thigh. Epidermal nevi often favor theextremities rather than the head and neck inwhat appears to be a dermatomal distribution(follows Blaschko’s lines). They may occur assingle lesions, but generally multiple lesionsare present arranged in a linear distribution.

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Figure 1.196. Linear verrucousepidermal nevus present at birthover the right torso of an infant.Note that this is similar to an epi-dermal nevus but has a more ver-rucous appearance and may bemore hyperpigmented.

Figure 1.197. Close-up of a linearverrucous epidermal nevus whichwas present only on the distal partof the left leg and foot of this oth-erwise normal infant. A localizedform (nevus verrucosus) usuallyconsists of a solitary lesion whichmay be grayish to yellowish-brownin color and warty, granular, orpapillomatous in appearance.

Figure 1.198. This infant pre-sented at birth with linear erosionswhich were open and appeared tobe traumatic. By the age of threemonths the erosions becamehypertrophic and hyperkeratotic.New areas developed over the pre-vious uninvolved areas of the skin,and later a verrucous appearancedeveloped. Skin biopsy diagnosiswas that of a classic linear verru-cous epidermal nevus. Centralnervous system, ocular, and longbone studies were normal.

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Figure 1.199. Nevus unius lateris is the term used when the lesions of epi-dermal nevus are extensive and systematized. It may present as a single lin-ear or spiral warty lesion or, at times, as an elaborate, continuous, orinterrupted pattern affecting multiple sites. When the scalp, face, or neckis involved, adnexal tissues such as the sebaceous glands may be affectedand become enlarged. For this form, the term linear nevus sebaceus may beused. Linear nevus sebaceus should not be confused with nevus sebaceus ofJadassohn, a distinct and unrelated disorder.In the linear nevus sebaceus syndrome, small linearly arranged verrucousyellow-orange nodules are seen on the face, neck, and limbs. Multiple seba-ceous nevi may be found on the scalp and there may be pigmented nevi onthe extremities. These lesions may be associated with ocular dermoids andmajor ophthalmic abnormalities (cloudy cornea and colobomata of the eye-lid, iris, and/or choroid). In the central nervous system there may beintracranial malformations which are associated with seizure disorders.Pigmentary and skeletal abnormalities may also be present.

Figure 1.200. Close-up of the face of the same infant as in Figure 1.199with the linear nevus sebaceus syndrome. Note the typical lesions in themidfacial area which extend from the forehead down into the lower jaw.Note that these are linear in distribution. Also note the dermoids of theeye. This infant developed seizures at the age of 10 days. The EEG wasgrossly abnormal and a diagnosis of cortical atrophy was made. The infantdied at the age of 2 months.

Figure 1.201. Linearepidermal nevi inanother infant whohad central nervoussystem abnormalitiesand seizures.

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Figure 1.202. Nevus sebaceus ofJadassohn is a hamartomatous lesionwith mixed tissue components thatmost commonly occurs on the scalpand face. These nevi are seen atbirth as flat or slightly elevated,well-circumscribed, sometimeselongated, orange-yellow to yellow-ish-brown plaques with a greasy orwaxy hairless surface. The lesionsare usually solitary, round or oval onthe scalp, or linear on the face andears. They vary from a few millime-ters to centimeters in diameter. Thecondition is not inherited but famil-ial forms may occur, are noted withequal frequency in males andfemales, and occur in all races.

Figure 1.203. Another example ofa nevus sebaceus of Jadassohn. Notethat this lesion is larger and not asraised as the nevus in the previousinfant. The lesions persist, enlargegradually, and malignant degenera-tion may occur in 10 to 15% oflesions generally during adolescenceor adult life. The most frequentchange is that of a basal cell carci-noma. Rapid enlargement or ulcera-tion of the nevus at any age maysuggest malignant change.

Figure 1.204. Nevus sebaceus ofJadassohn in the scalp. Note thatthis is a round, small lesion whichcould easily be missed on the initialphysical examination. Linear epi-dermal nevi and verrucous nevishould not be confused with nevussebaceus of Jadassohn which is a dis-tinct and unrelated disorder.

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Figure 1.205. An example of a nevus sebaceus ofJadassohn involving the left ear.

Figure 1.206. Note the raised lesions on the face (upperfigure) and similar lesions on the left shoulder and back(lower figure) present at birth in this infant who was other-wise normal. Skin biopsy diagnosis was that of nevoid,unclassified. This is a benign hamartomatous nevus inwhich the skin lesions are of different cell types and varyconsiderably in size and location.

Figure 1.207. Epidermolysis bullosa is an autosomallyinherited blistering and bullous disorder which is pre-sent at birth or becomes manifest soon after birth. Itcommonly affects the face, hands, feet, and variablythe limbs and trunk and is characterized by trauma-inflicted erosions (Nikolsky’s sign) and loss of the epi-dermis. As in this infant, the lesions are mostcommonly seen over the extensor surfaces and thereare broken bullae with underlying erythroderma.Epidermolysis bullosa occurs in three major inheritedforms: e. bullosa simplex (epidermolytic), junctional e. bullosa (letalis; gravis), and e. bullosa dystrophica(dominant and recessive forms) based on the presenceor absence of scarring, the mode of inheritance, andthe level of skin cleavage following minor trauma.

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VESICULOBULLOUS ERUPTIONS

Figure 1.208. In this infant with epidermolysis bullosa, pressure of the for-ceps blade at delivery has resulted in a positive Nikolsky’s sign. InNikolsky’s sign, firm stroking or rubbing of the skin will cause developmentof localized separation and tearing of the skin. In the Koebner phenome-non, minimal trauma (rubbing of the skin) may result in the formation ofblebs (vesicles and bullae). Vesicles are blistering lesions less than 0.5 cmin diameter, whereas bullae are blistering lesions 0.5 to 1 cm or more indiameter.

Figure 1.209. Bullous formation on thelip of the same infant as in Figure 1.208as a result of resuscitation.

Figure 1.210. In epidermolysis bullosasimplex, an autosomal dominant condi-tion, there is inadequate bondingbetween the epidermal and dermal layerswith separation of the skin at the basalcell and intraepidermal junction. Whenthe bullae break, the denuded areasresemble second degree burns but theulcerated vesicles and bullae resolvewithout scarring and heal rapidly.(Sometimes in severe cases there may bemild scarring.) Mucous membranes andnails are usually not affected.

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Figure 1.211. Note the bullae on the thirdand fourth toes and the extensive erosions(after rupture of the bullae) elsewhere onthe foot of this infant with the dystrophicform of epidermolysis bullosa. In dystrophicepidermolysis bullosa, the blister forms inthe papillary dermis below the basementmembrane. These areas heal gradually, leav-ing atrophic scars, keloids, and contrac-tures. Fingers and toes are bound together ifthere is loss of digits, and a mitten-like massmay form. Dystrophy of the nails may occur,leading to loss of the nails. This is an auto-somal recessive condition which affectsskin, mucous membranes and nails.

Figure 1.212. Epidermolysis bullosa in which the lesions were present atbirth. The feet are one of the most commonly affected sites because babieskick their feet. If the lesions are extensive, the infant is at risk for fluid andelectrolyte loss as well as infection.

Figure 1.213. Epidermolysis bullosa letalis(junctional epidermolysis bullosa, Herlitz’sdisease) in an infant with generalizedinvolvement of the skin. Note the massiveinvolvement of the lower extremities show-ing the denudation, scarring, and contrac-tures. In this form of epidermolysis bullosa,the skin separates in the lamina lucida of thedermal-epidermal junction and blisteringleads to mild atrophic changes. Junctionalepidermolysis bullosa is the most severe formof epidermolysis bullosa. It is characterizedby blistering and large erosions, mainly onthe buttocks, trunk, and scalp without scar-ring unless complicated by secondary infec-tion. Approximately 50% of these infantsdie within the first 2 years of life; some sur-vive into adulthood. Therefore, recently theterm “letalis” has not been used.

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Figure 1.214. In transient bullous der-molysis of the newborn, the lesions arepresent at birth or shortly thereafter.With the bullous appearance of thelesions, epidermolysis bullosa should beconsidered in the differential diagnosis,but the family history and rapid regressionof the lesions can confirm the diagnosis oftransient bullous dermolysis of the new-born. The sibling of this infant had simi-lar lesions at birth. In the figure on theleft note the large bullous lesions on the5th finger of the right hand. There weresimilar lesions on the left hand at birth.In the figure on the right note the healingof the lesions 5 days after birth.

Figure 1.215. This postmature infant is an exampleof a so-called “collodion baby.” Collodion babies areseen in postmaturity, lamellar ichthyosis, and con-genital ichthyosiform erythroderma. The term “col-lodion” is used because of the varnished (glisteningparchment-like) appearance of the skin which is sim-ilar to that resulting from collodion applied to theskin. This infant was postmature and the skinimproved rapidly.

Figure 1.216. Another example of a collodion baby isthis infant with lamellar ichthyosis who was bornencased in a membrane. In addition to the collodionappearance of the skin, note the ectropion (eversion ofthe eyelids with exposure of the palpebral fissure) andeclabium (eversion of the lips which causes inability tosuck) which occur as a result of the puckering of theskin, and the hyperkeratosis of the palms and soles.Mobility of the infant may be limited by the tightness ofthe membrane and also respiratory difficulty may occurdue to restriction of chest expansion until spontaneouspeeling begins in the first few days. The cutaneous cov-ering dries out and is gradually shed in large sheet-likelayers leaving a residual redness and hyperkeratosis. Lamellar ichthyosis is inherited as an autosomal reces-sive trait with two variants: the classic, more severe formof lamellar ichthyosis, and a milder erythrodermic vari-ant (non-bullous ichthyosiform erythroderma). (Levy,M., Moise, K.)

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SCALING DISORDERSDifferent abnormalities of the stratum corneum are included under the general heading “ichthyosis.” These con-ditions all involve faulty keratinization. The name is derived from the small plates of thickened epidermis yield-ing a pattern similar to the scales of a fish.


Figure 1.217. Another infant with a milder form oflamellar ichthyosis in which the parchment-likeappearance of the skin is not as generalized. The skindevelops widespread scaliness which may result in ageneralized scaly erythroderma. If scaling and erythro-derma are extensive, there may be excessive fluid lossresulting in hypernatremia, dehydration, and tempera-ture instability. These infants are also more susceptibleto infection.

Figure 1.218. Close-up of the face of the same infantas in Figure 1.217, showing the parchment-like appear-ance of the skin and the mild ectropion.

Figure 1.219. Close-up of the abdominal wall and thigh of the same infantas in Figures 1.217 and 1.218 with lamellar ichthyosis. Note the ichthyoticappearance of the skin, particularly in the upper abdomen, and the markedfissuring of the skin below as a result of spontaneous peeling. The underly-ing skin may be normal or may scale and form a new membrane. Lamellarscales are most prominent over the face, trunk, and extremities. Nailinvolvement is variable.

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Figure 1.220. Congenital ichthyosis may present as a hyperkeratotic form inwhich widespread scaling occurs frequently with underlying erythroderma.

Figure 1.221. In bullous congeni-tal ichthyosiform erythroderma(epidermolytic hyperkeratosis)there is a combination of bullouslesions, erythema, and desquama-tion. The presence of bullae ishighly characteristic of this disor-der. The blisters occur in crops andvary from 0.5 cm to several cm indiameter. They are superficial, ten-der, and when ruptured leave rawdenuded areas. The bullous lesionspresent at birth and result in theerythema and scaling as noted here.The differential diagnosis includesepidermolysis bullosa, toxic epider-mal necrolysis, and the differenttypes of ichthyosis.

Figure 1.222. The torso of thesame infant as in Figure 1.221showing the typical erythema anddesquamation following rupture ofthe bullous lesions. Improvement israpid in these infants and withhealing a generalized hyperkerato-sis may remain. This consists ofthick grayish-brown scales whichcover most of the skin surface espe-cially the flexural creases and inter-triginous areas which may showmarked involvement often withfurrowed hyperkeratosis.

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Figure 1.223. Another exampleof a milder form of the bullous con-genital ichthyosiform erythro-derma.

Figure 1.224. Harlequin fetus(congenital ichthyosis) is the mostsevere form of ichthyosis. It is anautosomal recessive conditionwhich has generally been regardedas being incompatible with life.There is a tight constrictingintegument with a leather-likeconsistency. The skin, in additionto being thick and rigid, is crackedand hard with deep crevices.Survival is rare, with death occur-ring as a result of respiratory insuf-ficiency due to thoracic rigidity,and infection. The harlequin fetusoften has unexplained fever andfailure to thrive.

Figure 1.225. Close-up of thehead and upper trunk of the sameinfant as in Figure 1.224. Note thethick, dry, rigid, hardened, crackedskin with deep crevices. The divi-sion of the thickened grey- to yel-low-colored skin into polygonal,triangular or diamond-shapedplaques by the deep reddish to pur-ple fissures has been said to simu-late the traditional costume of aharlequin. The skin has beenlikened to the bark of a tree, croc-odile skin, or Moroccan leather.

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Figure 1.226. A close-up view of the face and left upper extremity of thesame infant as in Figure 1.224 and 1.225. Note the marked ectropion result-ing from rigidity of the skin about the eyes with conjunctival redness andedema. There is eclabium with some cracking and fissuring at the corners asa result of the unyielding skin. The right hand is severely affected and hasresulted in gangrene of the fingers. The left hand has similar involvement.

Figure 1.227. The hands and feet of the same harlequin fetus asin Figures 1.224, 1.225 and 1.226 show severe deformities whichhave occurred as a result of the tight constricting integumentand compromise of the ectodermal structures, resulting ingangrene of the fingers and hypoplastic toes. The hands and feetare ischemic, hard, and waxy, often with poorly developed digitsand an associated rigid and claw-like appearance of the limbs.The nails may be hypoplastic or absent.

Figure 1.228. Another exampleof a harlequin fetus. Note theparchment-like skin, the ectro-pion, and eclabium. The mouthwith the eclabium sometimes has afish-like appearance. The unyield-ing skin has resulted in crackingand fissuring at the corners of themouth and the left hand is severelyaffected. This infant was treatedwith the oral administration ofetretinate and topical use ofEucerin™. Following use of thisapproach there has been prolongedsurvival in a few infants.

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Figure 1.229. The hands of thesame infant as in Figure 1.228. Notethe pale, waxy, firm appearance andpoorly developed digits which havebecome gangrenous. The nails maybe hypoplastic or absent.

Figure 1.230. The feet of the same infant as in Figure1.228 and 1.229 show the thick, rigid, hard, cracked skinand poorly developed toes with hypoplastic nails.

Figure 1.231. The CHILD syndrome isan X-linked dominant disorder (lethal tomales) which is characterized by congeni-tal hemidysplasia, with ichthyosiform ery-throderma, and limb defects. Thehallmark of the disorder is the sharp mid-line demarcation and the ipsilateralinvolvement of the skin and the extremi-ties. The face is spared, and limb defectsrange in severity from hypoplasia of digitsto complete agenesis of an extremity. Inaddition to the dermal and musculoskele-tal involvement, other organs may beabnormal (viscera and occasionally thecentral nervous system).

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Figure 1.232. Following a spontaneous vaginaldelivery in this term infant, desquamation of theskin was noted with the usual drying off in thedelivery room. No bullae or blisters were noted.There was superficial desquamation over about70% of the total body surface area. TheNikolsky’s sign was positive. The diagnosis of“peeling skin syndrome” was confirmed by skinbiopsy. This is an unusual congenital ichthyosiswhich is probably an autosomal recessive diseasecharacterized by lifelong peeling of the epidermiswith easy separation of the stratum corneum.

Figure 1.233. Another view of the same infant as in Figure1.232 with the “peeling skin syndrome.” This condition mustbe differentiated from non-bullous ichthyosiform erythro-derma. Histopathologic features include intraepidermal sepa-ration between the stratum corneum and stratum granulosum.There may be a biochemical marker of moderate generalizedaminoaciduria and low tryptophan levels.

Figure 1.234. The typical facies of hypohidrotic (anhidrotic)ectodermal dysplasia is seen in this infant. Note the alopecia,absent eyebrows and eyelashes, square forehead with frontalbossing, hyperpigmented wrinkles around the eyes, flattenednasal bridge, and large conspicuous nostrils. There are widecheek bones with depressed cheeks, thick everted lips, aprominent chin, and the ears may be small and pointed.These infants have a thin dry skin, decreased sweating,decreased tearing, and abnormal dentition. The nails aredefective in a large percentage of these patients in that theymay be thin, brittle, or ridged. If the absence of the sweatglands is generalized, they may have recurrent fever in highenvironmental temperatures.

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OTHER DERMATOLOGIC PROBLEMS


Figure 1.235. Radiograph of the face shows the typical hypoplasia of themaxillary alveolar processes and the lack of teeth (one of the hallmarks ofhypohidrotic ectodermal dysplasia). Ectrodactyly-ectodermal dysplasia-clefting (EEC) syndrome should be considered in the differential diagnosis.

Figure 1.236. Cutis verticis gyrata is anunusual disorder characterized by coarse fur-rowing, most commonly of the vertex of thescalp and posterior aspect of the scalp andneck. The vertex usually has anteroposteriorfurrows whereas on the forehead and occiputthey are transverse. The hair tends to besparse on top of the folds and more profuse inthe furrows. The entire scalp or only a smallpatch may have parallel folds of scalp skin fur-rowing with remarkable resemblance to theconvolutions of the brain.

Figure 1.237. A close-up of cutisverticis gyrata in the same infant as inFigure 1.236. The condition is causedby an increase in dermal collagen and,as a result, excessive skin may buckleand form furrows and ridges thatresemble gyri of the cerebral cortex.The condition may occur as a primarydisorder without other associatedabnormalities or it may be a manifes-tation of other pathology includingEhlers-Danlos syndrome, tuberoussclerosis, Apert’s syndrome, hyperpi-tuitarism, or pachydermomyositis.

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Figure 1.238. Neonatal lupus erythematosus is aunique variant of lupus erythematosus which occurs ininfants born to mothers with or having a tendency forsystemic lupus erythematosus, rheumatoid disease, etc.The majority of infants born to these mothers are usu-ally normal, but they may have a lupus-like rash andlupus-associated hematologic and serologic abnormali-ties. The cutaneous lesions appear from birth (in two-thirds of cases) until about 12 weeks of age. Themajority of infants have spontaneous resolution of thecutaneous lesions by about 6 to 12 months. In thisinfant note the diffuse erythema over the face andscalp, the scaly atrophic somewhat hypopigmented dis-coid lesions, and the alopecia which is a common find-ing in this disease. Cutaneous lesions of neonatal lupuserythematosus generally appear on the head and neck,extensor surfaces of the arms, and less frequently inother areas.

Figure 1.239. Sharply demarcated violaceous discoidlesions on the extensor surface of the forearm inanother infant with neonatal lupus erythematosus. Inneonatal lupus erythematosus about 15 to 30% ofinfants have congenital atrioventricular block, a com-plication which is usually irreversible with a high mor-tality rate (20 to 30% in the first few months of life.)During pregnancy the fetal ECG can be checked forheart block. Other systemic complications include car-diac malformations, hepatosplenomegaly, leukopenia,and thrombocytopenia. These infants have a positiveCoombs’ test and anti-Ro (SSA) antibodies are foundin 95% of affected mother-infant pairs. Neonatal lupuswas originally believed to be a transient disease but itis now apparent that continuing systemic involvementmay occur.

Figure 1.240. In erythema annularecentrifugum the lesions occur espe-cially on the body, develop quickly,and can last for weeks. The lesionsare erythematous and edematous, areof varying size and are partly ring-shaped in form. The centers of thelesions tend to fade and the lesionsmay spread out centrifugally andhave a slightly scaly edge. Theunderlying etiology of this conditionmay be autoimmune disorders in themother (such as lupus erythemato-sus), hypersensitivity to drugs, orfungal infection.

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Figure 1.241. Exfoliative erythroderma occurred inthis very small premature infant (birthweight 700 g) at4 weeks of age following a blood transfusion for anemiaat 3 weeks of age. He had a severe reaction and wascritically ill for several days. There was marked bloodeosinophilia and a diagnosis of graft-versus-host reac-tion was made. In acute graft-versus-host disease(GVHD) following transfusion, the findings include ageneralized erythematous maculopapular exanthemwith fine desquamation which later becomes a roughlamellar desquamation. There is no blister formation,and detachment of the nails may occur. Acute GVHDalso affects the liver and may give abnormal liver func-tion tests.

Figure 1.242. Close-up of the exfo-liative erythroderma in the sameinfant as in Figure 1.241. This infantdied at the age of 10 weeks, and at autopsy was found to have a pri-mary immunodeficiency syndrome.Cutaneous manifestations of acuteGVHD disease must be distinguishedfrom drug eruptions or viral exan-thems.

Figure 1.243. The diagnosis of a nasal dermoid in thisinfant was confirmed by biopsy. Dermoids are usuallypresent at birth and occur particularly along the linesof embryonic fusion. They are most common on thehead, especially around the eyes and the nose.Dermoid cysts may be attached to underlying struc-tures. In any midline nasal mass, intracranial extensionis common; thus, prior to removal of the mass,intracranial involvement should be excluded.

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Figure 1.244. The diagnosis of a hamartoma ofhair follicle origin was established by biopsy of themidline lesions of the nose in this infant.

Figure 1.245. Congenital self-healing reticulohis-tiocytosis (Hashimoto-Pritzker disease) is a rare dis-ease usually present at birth or within the first fewdays of life. It is characterized by solitary or multi-ple reddish-brown, pink, or purplish papulovesicu-lar lesions mainly on the scalp, face, trunk, andextremities. They tend to break down in the center,form ulcerated craters, and involute spontaneouslywithin 2 to 3 months leaving white atrophic scars.Although the course is benign and self-limiting, itis important to differentiate this condition fromhistiocytosis X and other histiocytic conditions.Diagnosis is confirmed by skin biopsy.

Figure 1.246. In juvenile xan-thogranulomatosis the lesions arepresent at birth. They are character-ized by solitary or multiple yellow toreddish-brown papules and nodules ofthe face, scalp, neck, and sometimesthe sublingual areas and the proximalportions of the extremities or trunk.The lesions may enlarge and becomebright yellow as they mature. Spon-taneous regression usually occurs inthe first year of life. Diagnosis is con-firmed by biopsy which shows histio-cytes of the non-Langerhans’ cells andthe presence of Touton giant cells.The lesions are harmless but shouldnot be overlooked since there is anapparent association with neurofibro-matosis. (Kenny, J.)

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Figure 1.247. Skin lesions, present at birth in this infant withhistiocytosis X, on biopsy showed the typical finding of the presenceof histiocytes of the Langerhans’ cell type and eosinophilia. Theterm “congenital histiocytosis” includes Letterer-Siwe disease,Hand-Schüller-Christian disease, and eosinophilic granuloma.These conditions are now grouped together as histiocytosis X.

Figure 1.248. Chest radiograph in an infantat the age of 3 days with histiocytosis. Notethe infiltrative coin-like lesions which aretypical with pulmonary involvement in histi-ocytosis X. The presence of pulmonary andother systemic involvement worsens theprognosis.

Figure 1.249. In this infant with congenitalgeneralized fibromatosis, on the left note thelesion on the nose and the purplish area sur-rounded by a pale halo above the left eye-brow. On the right is a close-up of the lesionson the back of the same infant. The infantsmay also have firm hard nodules in the skinand subcutaneous tissue. In congenital gener-alized fibromatosis it is necessary to check thelong bones and lungs for systemic involve-ment.

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Figure 1.250. Radiograph of the long bones in another infant with congen-ital generalized fibromatosis showing the areas of erosion due to the presenceof generalized fibromata.

Figure 1.251. Chest radiograph showing extensivepulmonary involvement in an infant with congenitalgeneralized fibromatosis. The presence of pulmonaryinvolvement signifies a poor prognosis.

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87

Chapter 2Perinatal InfectionThe immediate and long-term effects of perinatal infection are a major problem throughout theworld. Perinatal infection is relatively common among the over 4 million births per year in theUnited States but the incidence is dependent upon the organism. One percent of newborn infantsexcrete cytomegalovirus. Fifteen percent are infected with Chlamydia trachomatis; one-thirddevelop conjunctivitis and one-sixth, pneumonia. One to eight per 1,000 live births develop bac-terial sepsis. In utero or perinatal infection with herpes simplex virus, Toxoplasma gondii and vari-cella-zoster virus occurs in about 1 per 1,000 live births and the sequelae may be severe. In-uteroacquired infection may result in resorption of the embryo, abortion, stillbirth, malformation,intrauterine growth retardation, prematurity, and the numerous untoward sequelae associatedwith chronic infection. Infection acquired at or soon after birth may lead to death or persistentpostnatal infection. Some infections may be inapparent at birth and present years later with signs(e.g., choreoretinitis of T. gondii, hearing loss of rubella virus, and immunologic defects of HIV).Early diagnosis and aggressive treatment of infection during pregnancy may lower the associatedmorbidity and mortality rates substantially.


Figure 2.1. Staphylococcal furunculosisdeveloped in this infant at the age of 6 days. AGram stain of the material in the lesionsshowed numerous polymorphonuclear leukocytesand gram-positive cocci. The culture grewStaphylococcus aureus. Included in the differen-tial diagnosis of these lesions are transientneonatal pustular melanosis and herpes.

BACTERIAL INFECTION

Figure 2.2. Staphylococcal pyoderma of the neck in an infant atthe age of 8 days.

Figure 2.3. Bullous impetigo (pemphigus neonatorum) in a newborn infantat the age of 6 days. This infection may occur as early as the second day or aslate as two weeks of life and may demonstrate both bullous and impetiginouslesions. It is most commonly due to a staphylococcal infection but, on occa-sion, is caused by Streptococcus. The lesions are more common in moist, warmareas such as the axillary folds of the neck or the groin and present as super-ficial bullae which are wrinkled, become flaccid, and rupture easily producingulcers which become crusted. Note the impetigo of the umbilical area.

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Figure 2.4. Scalp abscesses developedat 12 hours of age. Note the distribu-tion over the area of the caput. Insuch an infant the diagnoses of herpessimplex and Staphylococcus aureusinfection should be considered.Cultures from the lesions and theblood in this infant were positive forStaphylococcus aureus. The motherdeveloped fever 24 hours postpartum,and Staphylococcus aureus was culturedfrom the episiotomy.

Figure 2.5. This nosocomial scalpabscess developed in a prematureinfant (birthweight 1000 g) at the ageof 12 days. Staphylococcus aureus wascultured from both the abscess and theblood. The parietal area is the mostfrequent site for scalp abscess. Scalpabscesses frequently occur with theuse of repeated scalp vein infusions.

Figure 2.6. Postauricular scalp abscessin an infant due to Staphylococcusaureus infection as a result of forcepsapplication.

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Perinatal Infection 89


Figure 2.7. Healing staphylococcal abscess resultingfrom application of forceps during delivery. These usu-ally are noted over the parietal area. Skin abrasionscaused by application of forceps or scalp electrodes forfetal monitoring may become secondarily infected,resulting in the development of a scalp abscess.

Figure 2.8. Staphylococcal parotitis in atwo week old infant. Note the swelling overthe parotid area.

Figure 2.9. Acute staphylococcal dacryocystitis devel-oped in this neonate at the age of 2 weeks. Note thatin addition to the swelling over the lacrimal gland,there is conjunctival inflammatory change.Staphylococcal infection is the most common cause ofthis infection, but it may occur with infection byStreptococcus or Neisseria.

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Figure 2.10. Breast abscess in aneonate due to Staphylococcus aureus.In the left figure note the infection asseen in the lateral view. A close-up ofthe abscess is shown in the right figure.

Figure 2.11. Mastitis of the rightbreast with abscess formation and cel-lulitis of the chest.

Figure 2.12. On the left note the nor-mal physiologic engorgement of thebreast. On the right there is a mastitissecondary to Escherichia coli infection.Although the most common cause ofmastitis in the neonate is staphylococ-cal infection, other organisms may beresponsible.

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Figure 2.13. This large abscess over the xiphoid areadeveloped at the age of 10 days in a neonate who hadmethicillin-sensitive Staphylococcus aureus sepsis.

Figure 2.14. Staphylococcal omphalitis and funisitisin a neonate. The erythema of the skin surroundingthe umbilical cord is due to omphalitis. Funisitis repre-sents infection of the umbilical cord per se. Note theredness of the umbilical cord.

Figure 2.15. Nosocomial infection causing an abscessof the right wrist and cellulitis of the 4th finger in apremature infant (birth weight 1100 g) at the age of 35 days. This infant had methicillin-resistantStaphylococcus aureus bacteremia. There was no osseousinvolvement. The infant was successfully treated withvancomycin.

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Figure 2.16. Acute osteomyelitis of thedistal end of the right femur presentingwith marked swelling of the right kneejoint. Blood culture was positive forStaphylococcus aureus. The diagnosis ofosteomyelitis must be excluded in anyneonate with a swollen joint or who isreluctant to move a limb spontaneously.Neonatal osteomyelitis may occur 1) bydirect inoculation, 2) by extension frominfection in surrounding soft tissues (e.g., infected cephalhematoma), 3) bytransplacental extension from maternalbacteremia (e.g., congenital syphilis), and4) by blood-borne dissemination inneonatal septicemia (the major cause ofneonatal osteomyelitis by metastatic seed-ing of the skeletal system through thenutrient arteries).

Figure 2.17. Lateral view of the rightknee joint in the same infant demon-strates the marked joint swelling. Thehips or knees or both are involved in 70%of cases of neonatal osteomyelitis. Thehigher incidence of sepsis in prematureinfants may contribute to the muchhigher incidence of osteomyelitis in pre-mature infants.

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A CB

Figure 2.18. Radiographs of the sameinfant showing, left to right, A)marked swelling of the soft tissues andthe joint with little evidence of bonychange, B) two days later note earlychanges at the distal end of the femur,and C) marked improvement after 1month of treatment. Unlike older chil-dren in which radiologic changes aredelayed for several weeks, in the neonatechanges of bone destruction are almostalways present by the 7th to 10th dayof illness. Capsular distention orwidening of the joint is common. Thereparative phase begins within 2 weeksafter onset of infection, and the entireprocess from the first signs of rarefactionto restoration of the cortical structuresmay last no longer than 2 months.


Figure 2.19. Osteomyelitis of the proximal end of the right femur withmarked bony changes. Note the marked increase in the size of the hipjoint. This again demonstrates that joint swelling may be the first indica-tion of the development of osteomyelitis. The reason for the commoninvolvement of joints in the neonatal period is that sinusoidal vessels,termed transphyseal vessels, connect the two separate circulatory systemsseen in the bones of older children (the metaphyseal loops which derivefrom the diaphyseal nutrient artery and the epiphyseal vessels whichcourse through the epiphyseal cartilage canals). With skeletal maturationthe transphyseal vessels obliterate (8 to 18 months) and the epiphysealand metaphyseal systems become totally separate.

Figure 2.20. Neonatalosteomyelitis due to Proteusmirabilis infection. AlthoughStaphylococcus aureus is themost common etiologic agentof osteomyelitis in the neonate,many other organisms suchas group B Streptococcus, E.coli, Klebsiella, Salmonella andCandida have been implicated.

Figure 2.21. Osteomyelitis usually occurs in the long bones,but in the neonate frequently occurs in other bones such as theclavicle and ribs. This infant demonstrates inflammation andswelling over the right clavicle due to a staphylococcalosteomyelitis.

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Figure 2.22. Severe scalp defect occurring as a resultof an underlying staphylococcal osteomyelitis of theskull.

Figure 2.23. This infant presented with fever,lethargy and poor feeding at 4 days of age. He thendeveloped a generalized rash which resembled scar-latina. Blood culture was positive for a Staphylococcusaureus phage type which produces an erythrogenictoxin, hence the appearance of the rash.

Figure 2.24. Close-up of the rash in the same infant.

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Figure 2.25. This infant who devel-oped a mild scalded skin syndrome(toxic epidermal necrolysis; Ritter’sdisease) at the age of seven days hadStaphylococcus aureus sepsis (methi-cillin-sensitive). Note the large bul-lae at this very early stage of thestaphylococcal scalded skin syn-drome. This is rapidly progressive.The skin is erythematous with vesic-ular and bullous formation, andthere is widespread wrinkling andloosening of the epidermis, which re-sults in the scalded skin appearance.

Figure 2.26. In the same infantthere is rupture of the bullae.These infants present with thetypical Nikolsky’s sign in that thereis skin exfoliation which peels ontouch.

Figure 2.27. In this infant withthe staphylococcal scalded skinsyndrome, the bullous lesions haveruptured, resulting in the scaldedskin appearance. Staphylococcalscalded skin syndrome is alsoknown as Ritter’s disease.

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Figure 2.28. This infant had rapid progressive toxicepidermal necrolysis. The face is usually affected firstand progression may rapidly become generalized. Thiscondition is most commonly due to phage group typeII staphylococci, and the toxin from the organismcauses the severe exfoliative dermatitis which resultsin the systemic manifestations of fever, instability andwater loss.

Figure 2.29. The body, buttocksand lower extremities of the sameinfant showing the very extensive“scalding” of the skin.

Figure 2.30. This infant has necrotiz-ing fasciitis of the abdominal wall,which is a rapidly progressive acutenecrotizing infection of the skin, subcu-taneous tissue, muscle, and fascia.Necrotizing fasciitis usually presents asan area of cellulitis with fever, redness,and edema. It rapidly progresses tocentral patches of bluish discolorationfollowed by ulceration, gangrene, andtoxicity. Necrotizing fasciitis is a surgicalemergency as it is rapidly fatal if nottreated aggressively by widespreadincision and debridement. Infection inthis condition has been associated withStaphylococcus aureus, anaerobic Strepto-coccus, Bacteroides and Proteus.

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Figure 2.31. Staphylococcal sepsis resulting in gangrene of theright foot and left big toe. Umbilical catheters were not used inthis infant.

Figure 2.32. Forty-eight hours after circumcision atthe age of 3 days, this infant developed cellulitis andinflammatory change of the glans and penis. There wasthrombocytopenia and positive blood culture forStaphylococcus aureus.

Figure 2.33. Bilateral prepatellar bursitis in a very active neonate whoabraded her knees. Aspiration of the purulent material yielded group BStreptococcus. The knee joints, per se, were not affected. This complicationis extremely rare. (Edwards, M.)

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Figure 2.34. Marked cellulitis of the right side of the facein a neonate with group B streptococcal infection. Therewas a positive blood culture for group B Streptococcus.

Figure 2.35. Beta hemolytic streptococcal sepsis israre in neonates. This infant had a generalized scarla-tiniform rash. Cultures were positive for group AStreptococcus (bacitracin-sensitive).

Figure 2.36. In the same infant as in Figure 2.35, notethe marked desquamation of the skin 1 week later.This is similar to the typical desquamation in scar-latina in older children.

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Figure 2.37. This infantwith streptococcal sepsisdeveloped cellulitis over aprominent xiphoid process.

Figure 2.38. Gonococcalophthalmia neonatorumin an infant at the age of 4days. With prophylaxisthis disease is rarely seentoday. The most commoncause of neonatal oph-thalmia at the presenttime is staphylococcalinfection.

Figure 2.39. Close-up of an infant with marked puru-lent discharge caused by gonococcal ophthalmianeonatorum. Even with Credé’s method, occasionallya breakthrough of gonococcal infection does occur ininfants in that they may develop a mild conjunctivitisat the age of 7 to 14 days which is positive for Neisseria.

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Figure 2.40. Gonococcal ar-thritis of the left ankle joint inan infant at the age of 2 weeks.As in the adult, neonatal gono-coccal infection generally affectsthe large joints (knee, ankle,etc.).

Figure 2.41. Haemophilus influenzae cellulitis of theprepatellar area in a neonate. Note the violaceous dis-coloration. This is very typical of Haemophilus influen-zae cellulitis. The infant may be very ill with this typeof infection. Cellulitis can occur as a result of infectionwith many other organisms.

Figure 2.42. In this infant withcongenital listeriosis, skin lesionswere present over the entire bodyat birth. These increased innumber. The infant had severerespiratory distress and had acongenital pneumonia due tolisteriosis. The amniotic fluid wasbrown in color and Listeria mono-cytogenes was cultured from bothskin lesions and the blood.Chocolate-brown amniotic fluidis typically seen in listeriosis. Ifmeconium staining is reported inpremature infants of less than 32weeks gestation listerial infectionmay be the cause. A good site forculture of the organism is themeconium or stool.

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Figure 2.43. Close-up of theskin lesions of the same infant asin Figure 2.42. Note the mac-ules, papules and vesicles. In thesepticemic form of listeriosis, acutaneous eruption of miliaryabscesses resembling papules,pustules, or papulo-pustules mayoccur over the entire body witha predilection for the back.Culture of the lesions, theblood, or cerebrospinal fluidusually reveals Listeria monocyto-genes as the offending agent.

Figure 2.44. Chest radiograph in an infant whopresented with severe respiratory distress from con-genital pneumonia due to congenital listeriosis. Theradiograph is not specific in that there may be peri-bronchial to wide-spread infiltration. In long-stand-ing cases a coarse mottled or nodular pattern may bepresent.

Figure 2.45. CT scan of a neonate who had listerial meningitis.Note the loss of morphology due to loss of white and gray matter dif-ferentiation. The villi are flattened out and there is evidence ofsome atrophy with fluid in the subarachnoid spaces. Listerial menin-gitis may present with early-onset disease (up to 7 days of age) usu-ally associated with serotype I or late-onset disease (mean age 12 to14 days) usually associated with serotype IV. Prognosis is poor inearly-onset disease as seen in this infant. Note that late-onset liste-rial infection presents with fever in 90 to 95% of cases whereashypothermia is much more common in other neonatal infections.

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Figure 2.46. Histologic sectionof the liver shows the typicalnecrotic lesion of granulomainfantisepticum. Human liste-riosis is characterized by the for-mation of miliary granulomasand focal necrosis or suppura-tion in the affected tissues.Listeria organisms are detectablein the necrotic foci. Theselesions of granuloma infantisep-ticum are classically observed ingeneralized listeriosis. Massiveinvolvement of the liver is pre-dominant, but the lesions arealso seen in the spleen, adrenalglands, lungs and throughoutother tissues.

Figure 2.47. This term infant developed cellulitis of the upperchest and back at the age of 9 days. Note the very definite lineof demarcation in the chest. At the same time the infantdeveloped a purplish area over the infraclavicular and scapularareas. A sepsis evaluation was performed and cultures werepositive for a gram-negative infection due to Paracolobactrum.(Paracolobactrum is in the same group as Escherichia, differingin that it has delayed fermentation of lactose. At the presenttime, it is included in the Salmonella-Arizona group ofEnterobacteriaceae.) Prior to the report of the blood culture,differential diagnosis included erysipelas, as the line of demar-cation was slightly raised, and gram-negative sepsis due toAchromobacter.

Figure 2.48. A close-up of the lesion 1 day later in thesame infant as in Figure 2.47. The lesion is moreviolaceous in color and the diagnosis of fasciitis wasmade.

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Figure 2.49. In the same infant as in Figure 2.47 and2.48, the infraclavicular lesion broke down with severedamage to the underlying subcutaneous tissue andmuscle ulceration developed; this healed with scarringover time. Note the lesion at the age of 37 days.

Figure 2.50. The same infant as in Figure 2.47 to 2.49at the age of 57 days showing the lesions with break-down and healing on the back.

Figure 2.51. Typical “fried egg” appear-ance (necrotic center with surroundinginflammation) of skin lesions associatedwith Pseudomonas infection are seen inthis 4-day-old neonate. This lesiondeveloped at the site of a Vitamin Kinjection. Pseudomonas aeruginosa is usu-ally a cause of late-onset disease ininfants who are presumably infected viaequipment, aqueous solutions or, onoccasion, the hands of health care per-sonnel. The same infant with Pseudomonas infec-tion shows healing at the age of ninedays. Note the necrotic center. Theselesions are indolent and slow healing.

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Figure 2.52. This infant withPseudomonas aeruginosa sepsis andmeningitis was acutely ill and hadconvulsions. He developed multi-ple skin lesions at the age of 12days. Convulsions were controlledwith difficulty and the infant diedone week later. Although it is veryuncommon, Pseudomonas conjunc-tivitis (a very purulent conjunctivi-tis) may be a devastating diseaseand if not promptly recognized andtreated there may be rapid progres-sion to septicemia, shock, anddeath. Pseudomonas sepsis may alsocause purpura fulminans (thrombo-cytopenia, and clinical and lab-oratory signs of disseminated intra-vascular coagulopathy).

Figure 2.53. Close-up view of thetypical lesions in the same infant.The cutaneous eruption inPseudomonas infection consists ofpearly vesicles on an erythematousbackground, which rapidly becomepurulent green or hemorrhagic.When the lesion ruptures, a cir-cumscribed ulcer with a necroticbase appears and may persist sur-rounded by a purplish cellulitis.

Figure 2.54. The typical lesionswere also present on the soles of thefeet. These lesions should not beconfused with the lesions ofsyphilitic pemphigus.

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Figure 2.55. These gangrenouslesions associated with bacteremiacaused by Pseudomonas aerugi-nosa demonstrate how devastat-ing this infection can be.

Figure 2.56. The typicalopisthotonic appearance in aninfant with neonatal tetanus.Note the head retraction, arch-ing of the spine, and the hyper-extension of the extremitiesresulting in a rigid posture.Neonatal tetanus is caused bygram-positive anaerobic spores ofClostridium tetani, present in thesoil and in animal and humanfeces. Infection usually occurs aftercontamination of the umbilicalstump that may result fromunsanitary delivery or uncleanhandling of the cord. In develop-ing countries the incidence ofneonatal tetanus remains high.

Figure 2.57. This infant withneonatal tetanus has the typicaltrismus giving rise to the risussardonicus and tetanic spasm.Especially note the hands. Risussardonicus is a grinning expres-sion produced by spasm of thefacial muscles.

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Figure 2.58. This infant with an omphalocele was delivered by a lay mid-wife under poor hygienic conditions. He developed tetanus at the age of 4 days and shows the typical risus sardonicus. (Cabrera-Meza, G.)

Figure 2.59. Close-up view of the face of the same infantas in Figure 2.58 showing the risus sardonicus. Note theexcess secretions. (Cabrera-Meza, G.)

Figure 2.60. Lateral radiograph of a skull. Note the gas ina scalp abscess caused by a localized infection by a gas-forming organism.

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Figure 2.61. Note the desquamation of the palms and solesin a neonate. There were no other dermatologic lesionselsewhere. The VDRL (Venereal Disease ResearchLaboratory) test showed maternal blood to be reactive witha titer of 1:32 and the infant had a titer of 1:1024. Lesionson the palms and soles should be considered syphilitic untilproven otherwise. The spectrum of cutaneous lesions whichoccur in 30 to 40% of infants with congenital syphilis canbe extremely variable. There may be mild desquamation,annular or circinate lesions or vesiculobullous manifestations.

Figure 2.62. Desquamation on thepalms and soles with no rash ordesquamation elsewhere is very sug-gestive of congenital syphilis. Thepalms and soles may be fissured anderythematous and, as a result of sub-cutaneous edema, may have a shinyappearance.

Figure 2.63. Syphilitic pemphigusshowing the large vesiculobulloushemorrhagic lesions on the soles ofboth feet. These lesions are rela-tively rare but, especially when seenon the palms and soles, are highlydiagnostic of this disease. Thelesions may contain a cloudy hem-orrhagic fluid that teems withorganisms and is highly contagious.With bullous lesions such as these,other dermatologic diagnosesshould be excluded (bullousimpetigo, epidermolysis bullosa,congenital bullous ichthyosiformerythroderma, etc.).

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Figure 2.64. A close-up of thefoot of the same infant as inFigure 2.63 shows both bullae andulcerated areas on the sole.

Figure 2.65. The same infant as in Figure 2.63 had bullae andulcerations on the palms of both hands. When bullae rupture,they leave a denuded area that can undergo extensive macer-ation and crusting. It is unusual to see the bullae at birth as themajority have ruptured in utero.

Figure 2.66. This infant withcongenital syphilis had vesiculob-ullous hemorrhagic lesions whichruptured in utero and presentedwith the typical raw, hemorrhagicappearance of the palms and solesat birth. These lesions are highlyinfectious.

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Figure 2.67. The raw hemorrhagic appear-ance of the lesions on the soles of the sameinfant as in Figure 2.66. In general, the moreflorid the manifestations of congenital syphilisat birth, the worse the prognosis.

Figure 2.68. Characteristic circinate lesionsinvolving the distal forearm in congenitalsyphilis. Syphilitic pemphigus of the palm isalso present. The cutaneous manifestationsmay appear as large round or oval maculopapu-lar (circinate) lesions. These are comparable tothe lesions seen in secondary syphilis in theadult. It is unusual for them to be present atbirth as they usually present between the age of3 to 6 weeks in infants with congenital syphilis.

Figure 2.69. Circinate lesions and syphiliticpemphigus of the soles present at birth in thesame infant as in Figure 2.68.

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Figure 2.70. Marked hepatosplenomegaly in an infant withcongenital syphilis. Hepatomegaly occurs in 50 to 60% ofaffected infants. It is frequently associated with jaundice, ane-mia, splenomegaly and ascites. In the spectrum of congenitalsyphilis there may be no clinical signs of disease at birth orthere may be many clinical manifestations which includeintrauterine growth retardation, skin manifestations, hepa-tosplenomegaly, jaundice, anemia, thrombocytopenia, andosseous changes.

Figure 2.71. This infant with massive ascites secondary tocongenital syphilis associated with respiratory distress,improved dramatically after abdominal paracentesis. Thisdegree of ascites caused dystocia, necessitating a cesarean delivery.

Figure 2.72. In an infant aged 6 weeks with the typical find-ings of congenital syphilis, note the circinate lesions over theforehead, the excoriation at the nose due to rhinitis, and thecheilitis at the corners of the mouth. Rhinitis (“snuffles”) usu-ally appears between the 2nd to 6th week of life and is theresult of ulceration of the nasal mucosa. When the ulcerationis deep enough to involve the cartilage of the nasal bone, thearchitecture is destroyed, thus giving rise to the classic saddlenose deformity. Mucous membrane patches are seen in approx-imately one-third of infants with congenital syphilis. At themucocutaneous junctions these lesions tend to weep and maycause fissures (cheilitis) which often extend from the lips in aradiating fashion over the surrounding skin. When deep, theselesions may leave residual scars (rhagades).

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Figure 2.73. This infant developed a skin rash of congenital syphilis atthe age of 7 weeks. These annular and circinate lesions healed but leftareas of hypopigmentation. This type of cutaneous lesion, rarely presentat birth, generally develops as a dark pink or red rash from the age of 3 to6 weeks; it gradually fades to a coppery-brown lesion which disappearsover a period of 1 to 2 months but often leaves an area of hyperpigmen-tation or hypopigmentation. The lesions may occur on any part of thebody, but are usually most pronounced on the face and the dorsal surfaceof the trunk and legs.

Figure 2.74. Congenital syphilitic chorioretinitis ofthe right eye. Note the abnormal scarring in the mac-ular area. Optic atrophy, if present, is usually seen inconjunction with neurosyphilis. These findings areextremely rare in congenital syphilis. Other stigmata ofcongenital syphilis present much later. The deciduousteeth of children with early congenital syphilis areprone to caries but show no other abnormality. Thestigmata of Hutchinson’s triad (Hutchinson’s incisors,interstitial keratitis, and eighth nerve deafness) occurlater. Dental changes of Hutchinson’s incisors andMoon’s (mulberry) molars occur with secondarydentition.

Figure 2.75. Condylomata lata presenting at the ageof 6 weeks in an infant with congenital syphilis. TheRPR (rapid plasma reagin) was negative at birth but at6 weeks was reactive with a titer of 1:512. The infantwas anemic with a hemoglobin of 4.8 mg/dL, three-plus hematuria, CSF (cerebrospinal fluid) pleocytosis,and a positive CSF VDRL. Lesions responded to peni-cillin 3 days after treatment was initiated. Condylomatalata appear as flat or raised, moist, wart-like cutaneouslesions, grayish-pink in color. They are smooth, roundor oval, and wide-based, often mushroom-like, lesionswhich may be single or multiple. They occur in moistareas, especially the anogenital regions. They healwithout scarring and are highly infectious and must bedifferentiated from condylomata accuminata which arecovered by digitate vegetations.

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Figure 2.76. Dactylitismay be a rare finding incongenital syphilis. Notethe spindle-shaped appear-ance of the fingers. This is arare form of osteochondritisof the small bones of thehands and feet which usu-ally appears between 6months and 2 years of age.It is commonly found inthe metacarpals, proximalphalanges of the hands, andthe metatarsals. The swell-ing gives rise to little painor discomfort.

Figure 2.77. Radiograph of the chest in an infant at age7 days. The interstitial pneumonia (pneumonia alba) ofcongenital syphilis is present. This is characterizedpathologically by yellowish-white, heavy, firm andgrossly enlarged lungs. Note the osseous changes of con-genital syphilis at the proximal ends of the humeri.

Figure 2.78. Radiograph of an infant with cogenital syphilis showingmassive ascites and growth arrest lines at the ends of long bones.

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Figure 2.79. This radiograph in the same infant asin Figure 2.78 shows growth arrest lines in the lowerextremity long bones. The growth arrest lines at theends of the long bones are a nonspecific finding inthat they signify interference with growth of thefetus in utero and, although commonly seen in con-genital syphilis, may occur in many other con-ditions such as congenital viral infections anderythroblastosis fetalis.

Figure 2.80. Growth arrest linesfound in the upper extremities in aninfant with congenital syphilis.Growth arrest lines show an enhancedzone of provisional calcification(radiopaque band) which is associatedwith osteoporosis immediately belowthe dense zone. The growth arrest linemay be smooth or serrated. A serratedappearance is known as Wegner’s sign.

Figure 2.81. Radiograph of the upperextremity of an infant with congenitalsyphilis showing growth arrest lines,periostitis, and fracture of the ulna.Periostitis is seldom visualized at birthbecause of lack of sufficient calcifica-tion at that time to cast a shadow. It ismore commonly seen at 4 to 6 weeks ofage and, at that time, must be distin-guished from that seen in healing rick-ets, child abuse, and infantile corticalhyperostosis. As the bone is more frag-ile with syphilitic infection, bonetrauma is more common.

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Figure 2.82. This radiograph demonstrates the lack of ossification cen-ters and the presence of growth arrest lines in the lower extremities of aterm infant with congenital syphilis. In congenital syphilis, some growthretardation may occur and it is not unusual to see a delay in appearanceof the ossification centers. The bony involvement tends to be multipleand symmetrical. Characteristically bony involvement is widespread andincludes the long bones, cranium and ribs. In most cases the osseouslesions are asymptomatic, but in some infants severe involvement maylead to subepiphyseal fractures with epiphyseal dislocation resulting in apainful pseudoparalysis (Parrot’s atrophy of newborn). This may mimicErb’s palsy.

Figure 2.83. Lower extremity radiograph showing characteristicerosions of syphilitic osteochondritis at the metaphyses of the dis-tal ends of the long bones. About 15% of infants with osteochon-dritis will show signs at birth. Ninety percent will show radiologicevidence of osteochondritis and periostitis after the first month oflife. In osteochondritis, there is increased widening of the epiphy-seal lines with increased density of the shafts, spotty areas of radi-olucency, and a resultant moth-eaten appearance.

Figure 2.84. Radiograph oflower extremities and rightupper extremity in this infantat the age of 7 days shows thetypical syphilitic osteochon-dritis and some periostealreaction, especially of thefemora. The osteochondritismay be present at birth ormay appear during the firstmonth of life. It is mostclearly seen in the longbones. The changes seen arefragmentation and apparentdestruction with mottledareas of radiolucency.

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Figure 2.85. Radiograph of the lower extremities of thesame infant as in Figure 2.84 at age 6 months. Note themarked alterations of the contour and thickness of thefemora. The cortex is thin and the medullary cavity isexpanded. The proximal end of the left femur is severelydamaged due to syphilitic osteomyelitis. This may presentas a syphilitic arthritis of the hip. In spite of these exten-sive changes, infantile luetic osteitis usually improvesremarkably.

Figure 2.86. Radiograph of the left humerus in an infant with congenitalsyphilis showing periostitis which was present at birth. The lesions ofperiostitis are usually diffuse and frequently extend over the entire lengthof the involved bone. It is first seen as a thin even line of calcification out-side the cortex of the involved bone; the lesions progress and eventuallyproduce calcification and thickening of the cortex. When severe, this leadsto a permanent deformity such as anterior bowing of the tibia (saber shins).Periostitis of the frontal bones of the skull, when severe, is responsible forthe flat overhanging forehead that may persist as a stigma of congenitalsyphilis in infancy.

Figure 2.87. Wimberger’s sign inan infant with congenital syphilisis recognized by radiolucency dueto erosion of the medial aspect ofthe proximal tibial metaphysis.Painless effusion in one or bothknees (Clutton’s joints) generallybecomes apparent between 8 to 15years of age, involutes sponta-neously and leaves no residualeffects.

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Figure 2.88. Higouménaki’s sign refers to periostitis of the clav-icle. This may be observed clinically or radiographically and is adiagnostic finding in congenital syphilis. Note also the periostitisof the ribs. Fracture of the clavicle with callus formation shouldbe a consideration in the differential diagnosis.

Figure 2.89. This infant at age 5 days developedfever, lethargy and poor feeding. On sepsis evalu-ation there was a pleocytosis of 120 WBCs in thecerebrospinal fluid indicative of meningoen-cephalitis. There was no evidence of cardiacinvolvement. The following day the infant devel-oped a generalized maculopapular rash and loosestools. He recovered without treatment. Stoolculture grew Coxsackie virus.

VIRAL INFECTION

Figure 2.90. Close-up view ofthe rash from the same infant asin Figure 2.89 with Coxsackievirus infection.

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Figure 2.91. This infant withcytomegalovirus infection has alow birthweight due to intrauter-ine growth retardation and showsthe “blueberry muffin” appear-ance, microcephaly, and abdomi-nal distention due to markedhepatosplenomegaly. Cytomega-lovirus infection is asymptomaticin approximately 90% of infectedinfants at birth. Of these, 5 to10% develop late-onset sequelaesuch as hearing loss, chorioretini-tis, mental retardation, and neu-rologic sequelae. The remaining10% may have mild to severe andoccasionally fatal disease.

Figure 2.92. A close-up of the head and face of the same infant as in Figure2.91 with microcephaly and the typical “blueberry muffin” lesions. Theselesions represent areas of dermal erythropoiesis. Cutaneous manifestationsinclude petechiae, purpura, “blueberry muffin” lesions (also seen in congen-ital rubella and other conditions), and vasculitis.

Figure 2.93. Another infantwith cytomegalovirus infectionshowing the typical “blueberrymuffin” appearance and jaundicesoon after birth. Note that thehead size is normal. Since mostinfections are asymptomatic,early diagnosis is only made inthe full-blown syndrome mani-fested by the appearance of jaun-dice within the first 24 hours oflife, hepatosplenomegaly, abdom-inal distention, anemia, thrombo-cytopenia, respiratory distress andneurologic changes.

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Figure 2.94. Close-up of the face of the same infant as inFigure 2.93 showing the typical “blueberry muffin” appearance.

Figure 2.95. This infant presented with areas of skin involvement on theextremities only. The lesions were asymmetrical in that they were present onthe right leg and left arm. Vasculitis, a less common manifestation of skininvolvement by cytomegalovirus infection, was confirmed by skin biopsy.

Figure 2.96. Lesions on the left arm of thesame infant as in Figure 2.95 show the vasculitis.

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Figure 2.97. Macular chorioretinitis which is typicalof cytomegalovirus infection.

Figure 2.98. Histopathologicsection of lung demonstratingthe typical “owl’s eye” appear-ance of cells infected withcytomegalovirus. Postnatallyacquired cytomegalovirus infec-tion can occur in infants withprimary immunodeficiencies orfollowing transfusion withcytomegalovirus-positive blood,etc. (Langston, C.)

Figure 2.99. “Owl’s eye” appear-ance in a histopathologic sectionof the kidney. Note the large cellu-lar size which results from anincrease in volume of both thenucleus and the cytoplasm. Thenuclear inclusion is located cen-trally and corresponds to the shapeof the cell. The nucleolus is usuallydisplaced peripherally in infectedcells. There is no necrosis of cells.(Langston, C.)

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Figure 2.100. Typical “owl’s eye”cells in the urine in an infant infectedwith cytomegalovirus. These cells aredistinctive and large, containingintranuclear and cytoplasmic inclu-sions. (Langston, C.)

Figure 2.101. Radiograph showing the postero-anteriorview of the skull of an infant infected with cytomegalovirus.Note the severe microcephaly and periventricular calcifica-tions. These calcifications demonstrate the marked enlarge-ment of both lateral ventricles. The changes were present atbirth.

Figure 2.102. Lateral radiograph of the skull of thesame infant as in Figure 2.101, again demonstratingmicrocephaly and periventricular calcifications.Periventricular calcification is seen most commonly incongenital cytomegalovirus infection whereas intracra-nial calcification in congenital toxoplasmosis is usuallymore generalized.

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Figure 2.103. CT scan of the head shows periventricular calci-fications and dilatation of the ventricles in an infant infectedwith cytomegalovirus. Intracranial calcification is present inabout 20% of infected infants and may be noted much earlier onCT than on initial skull radiographs.

Figure 2.104. The radiograph on the left shows the typical “celery stalk”appearance at the distal end of the femur. This appearance is commonlyfound in infants with congenital rubella, but on rare occasions can be seenin infants infected with cytomegalovirus. The radiograph on the right showsresolution of the abnormal findings at 1 month of age.

Figure 2.105. Herpetic lesions which appeared on thehand of an infant at the age of 5 days. The skin onlywas involved in this infant. There was rapid improve-ment on treatment with intravenous acyclovir.Infection may be acquired as the fetus passes throughthe birth canal. Thus, although skin lesions may bepresent at birth, the clinical picture of herpes in thenewborn is frequently that of an apparently well infantwho becomes symptomatic on the 4th to 8th day oflife. The spectrum of illness in herpes simplex virusinfection is broad ranging, from death or recovery withsevere central nervous system or ocular damage to mildor asymptomatic infection with apparent completerecovery.

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Figure 2.107. The same infant as in Figure2.106 developed a fresh crop of skin lesionsin the periumbilical area five days later whileon systemic therapy. This is not uncommonas herpetic lesions often appear in crops. Thisinfant also had herpes simplex encephalitis.Seventy percent of infants with neonatalherpes simplex virus infection have skinlesions, and 70% of these, if left untreated,will progress to systemic infection. Therefore,treat early.

Figure 2.108. Disseminated neonatal herpes simplex infec-tion in a premature infant who presented with skin involve-ment at age 6 days. The eruption is zosteriform and has beendescribed in infants with herpes simplex virus infection.Skin lesions that may occur include petechiae, purpura,zosteriform lesions, erythematous macular lesions thateventually develop vesicles within the macules, pustularerosions, and large bullae. About 80% of infected babiesdevelop skin lesions between 1 and 4 weeks of age.

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Figure 2.106. Herpetic lesions whichappeared on the skin at 7 days of age in aninfant who developed disseminated herpessimplex. Herpetic skin lesions are confluent,fluid-filled vesicles with an erythematoushalo around the base. These vesiclesbecome pustular after 24 to 48 hours andeventually become crusted or ulcerated.Most neonatal herpes simplex virus infec-tions (80%) are caused by Type II (genital)herpes virus, acquired either by ascendinginfection from the mother’s genital area tothe fetus or by spread during deliverythrough the birth canal of the infectedmother. Infection may be acquired bytransplacental spread (about 5% of cases) orby postnatal contact with other infants orpersonnel with oral herpes simplex virusinfections.


Figure 2.109. Herpetic skin lesionson the scalp in a premature infant(birthweight 1450 g). These lesionson the scalp appeared at the age of 4days. Occasionally the presenting partof the infant at delivery may be cov-ered by a diffuse edematous swellingresembling that seen in a caput suc-cedaneum. Rather than resolving dur-ing the first week, this may becomeboggy and necrotic with a resultantdraining sinus or eschar formationand tense irregular herpetic vesicles.

Figure 2.110. Disseminated neonatal herpes simplexinfection with skin lesions on the face and eyelids.This infant had keratitis and encephalitis. At timesconjunctivitis and keratoconjunctivitis may be seen asthe first presenting signs of neonatal herpes infection.This is subsequently followed by lethargy, poor feeding,temperature instability, jaundice, hepatosplenomegaly,and widespread herpes simplex dissemination.

Figure 2.111. Herpetic lesions pre-sent on the vulva following a breechpresentation. Note also the pres-ence of ecchymoses. Skin lesionsoccur most often on the scalp andface, the areas which are closest toand in longest contact with the cer-vical area from which infection istransmitted. Involvement of thecornea in vertex presentations andthe genitalia in breech presenta-tions is thus common in herpeticinfection.

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Figure 2.112. Herpetic skin lesions on the neck of a premature infant. Asimple Gram stain or Tzanck test would differentiate this from staphylococ-cal infection. The presence of multinucleated giant cells containing intranu-clear inclusions (balloon cells) are characteristic of viral infection.

Figure 2.113. This infantwith severe intrauterinegrowth retardation developedherpetic skin lesions andencephalitis. The infant wastreated with acyclovir andimproved, but the EEG andCT scan were grossly abnor-mal. One month followingtreatment he developed afresh crop of lesions on theright hand (vesicles withparonychia) with resolutionafter a second course of acy-clovir.

Figure 2.114. This infant presented with cardiovasular collapse and dis-seminated intravascular coagulopathy. She was well until age 12 days atwhich time she developed massive ecchymoses and petechiae with bleedingfrom the umbilical stump and rectum. There was rapid deterioration anddeath within 2 hours. Autopsy showed disseminated herpetic lesions. Theremay be hepatitis, pneumonia, coagulopathy with severe bleeding diathesis,and disease of the central nervous system (meningoencephalitis).

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Figure 2.115. Gross pathologicspecimen of the liver of thesame infant as in Figure 2.114showing herpetic lesions. Thedisseminated form of neonatalherpes simplex infection affectsthe visceral organs, chiefly theliver and adrenal glands. It mayalso involve the CNS and otherorgans, and results in a high mortality.

Figure 2.116. Parvovirus B19 infection resulting in intrauterine death in ahydropic stillbirth. Human parvovirus B19, the same virus that causes ery-thema infectiosum, has a special affinity for rapidly dividing cells, particularlyerythroblasts; therefore, an infection may result in profound anemia, hydropsfetalis, and death in the fetus. (Singer, D.)

Figure 2.117. Nonimmunehydrops fetalis due to par-vovirus B19 infection in a pre-mature infant born at 24 weeksgestation. Note the grosshydrops fetalis. Laboratoryanalysis was remarkable for ahemoglobin level of 1.4 g/dL,hematocrit of 4%, plateletcount of 10,000/mm3, andWBC count of 6000/mm3

(which, when corrected fornucleated red blood cells,showed a WBC count of 0).

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Figure 2.118. Radiograph of the same infant as in Figure 2.117 showingsevere hydrops fetalis. Note the massive soft tissue edema. In pregnantwomen with evidence of infection, maternal serum alphafetoprotein con-centration may provide a marker of fetal aplastic crisis. If the concentra-tion is increased, serial ultrasonography may be used to check thepossibility of fetal hydrops, and fetal sampling may indicate the severity offetal anemia. Treatment is by in utero transfusion to the fetus.

Figure 2.119. Patho-logic slide of parvo-virus B19 infection inerythroblasts. The viruscan be clearly seen onthe right in the elec-tron micrograph of anucleated red cell.(Singer, D.)

Figure 2.120. Congenital rubella in aterm infant with severe intrauterinegrowth retardation (birthweight 1300 g).Note the “blueberry muffin” appearance.Infected infants are usually born at term,but with low birthweight. In addition tothe “blueberry muffin” lesions there maybe thrombocytopenic purpura, hyper-bilirubinemia, hepatosplenomegaly,pneumonia, congenital cardiac defects(especially patent ductus arteriosus), eyedisorders, deafness, and meningo-encephalitis.

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Figure 2.121. Close-up view of the face of the same infant as inFigure 2.120. Note that the “blueberry muffin” lesions in this infantwere red rather than purple. In infants with fresh lesions the initialcolor is dark red but then changes to a bluish-red and dark blue colorover the course of a few days. The lesions are infiltrated maculesmeasuring 2 to 8 mm in diameter, are usually present at birth orwithin the first 24 hours, and new lesions are rare after 2 days.

Figure 2.122. This infant shows the typical “blueberry muffin” skinlesions. Histologically, “blueberry muffin” lesions reveal discrete dermalaggregates of relatively large nucleated cells and non-nucleated erythro-cytes. They are the result of dermal erythropoiesis (rather than true hem-orrhage). These infiltrative lesions, characteristic of viral infections in thefetus, are not unique to infants with congenital rubella but may be seen incongenital cytomegalovirus infection and congenital toxoplasmosis. Theyalso occur in erythroblastosis fetalis, congenital leukemia, etc.

Figure 2.123. The intrauterinegrowth retardation in congenitalrubella may be very striking as shownin this term infant with a birthweightof 860 g. The lesions of congenitalrubella may be few or numerous andgenerally occur on the head, trunk, orextremities. Many of the largerlesions tend to be raised. They usuallydisappear within 3 to 4 weeks (thelarger lesions more slowly than thesmall flat nodules).

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Figure 2.124. Although theinfant with congenital rubellawas of extremely low birth-weight, he nippled all feeds well.Note the presence of a few “blue-berry muffin” lesions.

Figure 2.125. Microphthalmia andcongenital cataract of the left eye in aninfant with congenital rubella.

Figure 2.126. Congenital rubellacataract.

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Figure 2.127. Corneal cloudingand prominence of left eye due to glaucoma in an infant with congenital rubella. One shouldsuspect glaucoma (buphthalmos)in any infant with a wide or cloudycornea.

Figure 2.128. Opisthotonos in an infant withcongenital rubella encephalitis. Viral culture fromcerebrospinal fluid was positive.

Figure 2.129. Adenopathy in an infant with congenital rubella.Enlargement of lymph nodes is uncommon in the neonate, but isdescribed in infants with other viral syndromes and in congenitalsyphilis where the epitrochlear glands especially are enlarged.

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Figure 2.130. Lateral radiograph of the skull in an infantwith congenital rubella. Note the very large anteriorfontanelle, prominent sutures and poor mineralizaton ofthe calvaria. Bony abnormalities are seen at birth in up to80% of infants with congenital rubella. These include thelarge anterior fontanelle and characteristic “celery stalkappearance.” These bony abnormalities also resemblethose of other fetal viral infections such ascytomegalovirus.

Figure 2.131. Anterior-posterior and lateral views of the skull in an infant with congenital rubella. Note the largeanterior fontanelle which extends into the metopic suture with poor mineralization of the calvaria.

Figure 2.132. Lower extremity radiograph showing the typical“celery stalk” appearance particularly at the distal end of thefemur. The longitudinal areas of radiolucency in the metaphysesof the long bones give rise to the radiographic appearance of“celery stalks.” This appearance is especially noted at the distalends of the femur or the proximal tibia.

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Figure 2.133. Lower extremity radiograph in an infant withcongenital rubella at 3 weeks of age. Note the radiolucent zonesparalleling the growth plates with residual irregular trabecularpattern. These are the nonspecific growth arrest lines whichmay be seen in any condition interfering with the growth of thefetus in utero.

Figure 2.134. Repeat radiographs of the same infant as inFigure 2.133 at 2 months of age showing the regression of thesechanges.

Figure 2.135. Radiograph compositethat demonstrates the types of bonychanges found in congenital rubella syn-drome: from left to right, A) “celerystalk” appearance; B) nonspecific growtharrest lines; C) a bony spicule at themedial condyle of the femur (an uncom-mon finding). All of these regress overthe first few months postnatally.

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A) B) C)

Figure 2.136. Children with congenital rubella may have anormal physical and neurodevelopmental outcome, although itis uncommon. Maternal rubella in the first 4 weeks of pregnancycarries a risk of congenital rubella of 50% (heart anomalies, eyedefects, hearing problems, etc.); between the 12th and 16thweeks of pregnancy the risk decreases to 2 to 6%; and betweenthe 18th to 20th weeks of pregnancy the risk is practically nil.Over 80% of babies with congenital rubella shed virus duringthe first month of life, and 5 to 10% (those severely affected),for one year.

Figure 2.137. Cicatricial skin lesions ofthe neck and upper back in an otherwisenormal infant following maternal vari-cella at 5 months gestation. These skinlesions are the most common finding aftermaternal varicella which presents in the1st and early 2nd trimester. Maternalinfection with varicella early in preg-nancy is a cause of fetal malformationsincluding reduction deformities of thelimbs (hypoplastic limbs and/or contrac-tures) and scars along the length of theaffected limbs. The infant may be smallfor gestational age and demonstrate fea-tures of central nervous system involve-ment (encephalomyelitis) and eye defects(microphthalmia, cataracts, and choriore-tinitis).

Figure 2.138. Congenital varicella in an infant which pre-sented at the age of 7 days. Mother developed varicella 10 daysprior to delivery. This infant had very few lesions and was notill. If the onset in the mother is within 4 days prior to or within48 hours after delivery, or if the onset in the newborn is between5 to 10 days after birth, the infant’s condition is usually moresevere. In these cases, varicella-zoster immune globulin (VZIG)should be given as soon as possible after birth.

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Figure 2.139. Another case ofcongenital varicella infection.Complications of congenitalvaricella may be disseminatedand fulminant with pneumo-nia, hepatitis, or encephalo-myelitis, and mortality is high.(Cabrera-Meza, G.)

Figure 2.140. This neonate with congenital varicella has exten-sive skin involvement. (Cabrera-Meza, G.)

Figure 2.141. Close-up of thesame infant as in Figure 2.140with congenital varicella, show-ing extensive skin lesions at dif-ferent stages. (Cabrera-Meza, G.)

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Figure 2.142. Anteroposterior (AP) skull radiograph in a child with con-genital toxoplasmosis. Note the diffuse areas of intracranial calcification.This is characteristic of toxoplasmosis and may be associated subsequentlywith hydrocephalus or microcephaly. Toxoplasma gondii infection is trans-mitted to the fetus by invasion of the bloodstream during the stage of mater-nal parasitemia, and may result in a wide spectrum of clinical signs andsymptoms. The fetus may be stillborn, born prematurely, or born at full term.

Figure 2.143. Lateral radiograph from the same child as inFigure 2.142, showing diffuse intracranial calcifications.Intracranial calcifications, in general, are diffuse and scat-tered in infants with congenital toxoplasmosis. Skull radi-ographs of infected infants frequently reveal this diffusepunctate intracranial calcification. The distribution of thecalcification in infants with congenital cytomegalovirusinfection is periventricular.

Figure 2.144. Lateral radiograph of the skullof a newborn infant infected with both toxo-plasmosis and cytomegalovirus. Diffuseintracranial calcifications are present.

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PROTOZOAL AND PARASITIC INFECTIONS


Figure 2.145. A CT scan in an infant with toxoplas-mosis at the age of 22 days, showing the scatteredintracranial calcification with some brain destruction.

Figure 2.146. The CT scan of another infant at the age of 1 monthshowed marked brain destruction with scattered intracranial calcifica-tions and hydrocephalus ex vacuo. The illness is usually apparent at birthin that the infant may have fever, poor feeding, vomiting and diarrhea,jaundice, and hepatosplenomegaly. There may be a maculopapular rashor “blueberry muffin” lesions as well as microphthalmia, cataracts, andchorioretinitis. The chorioretinitis is in the region of the macula and isseen in about 80% of infants with congenital toxoplasmosis.

Figure 2.147. CT scan of the same infant several days later with con-genital toxoplasmosis. Note the rapid progress with massive loss of brainparenchyma and multiple scattered areas of calcification. Peripheralwhite blood count was remarkable for 96% eosinophils. There werenumerous eosinophils in the cerebrospinal fluid. In toxoplasmosis, ane-mia, thrombocytopenia, and at times severe leukopenia may be present.The cerebrospinal fluid is xanthochromic, has an elevated protein level,and may contain erythrocytes and leukocytes.

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Figure 2.148. Pathologic specimenshowing a section of the brain from thesame infant as in Figure 2.147. Note thehydrocephalus and cortical necrosis pre-sent at autopsy.

Figure 2.149. This infant has neonatal tinea capitis(ringworm), which was diagnosed at the age of 3weeks. The condition is rarely seen in the neonate.Lesions are sharply outlined and ring- or disc-shaped,and there may be confluent areas of alopecia with areasof broken and brittle hair observed on an erythema-tous, scaling scalp (silvery scales). The diagnosis ofringworm of the scalp can frequently be made by thepresence of fluorescence under a Wood’s light (theaffected scalp appears green due to fluorescence of theinfected hairs) or by microscopic examination ofinfected hairs. In the neonate, the infection is usuallyproduced by Microsporon canis, M. audouinii, orTrichophyton tonsurans. The hair does not fluoresce in aTrychophyton tonsurans infection. (Levy, M., Moise, K.)

Figure 2.150. This infant pre-sented with scabies at the age of 17days. The mother had scabies. Thisparasitic infection is uncommon inthe neonate. The distribution ofthe lesions is different from that ofthe older child in that the face,head, and neck may be involved,especially if the mother is breast-feeding. There may be scabeticburrows, papules, and vesicularlesions. In addition there isinvolvement of the usual areassuch as the flexor surfaces of theextremities, the interdigital spaces,the groins and the axilla.

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Figure 2.151. A close-up of thehand in the same infant as in Figure2.150 showing the typical scabeticlesions. Primary lesions are burrows,papules, and vesicular lesions.Secondary bacterial infection caus-ing pustules may occur.

Figure 2.152. Typical candidal dia-per dermatitis. Note the symmetricdistribution of the rash with involve-ment of the intertriginous areas.Satellite lesions are often present.The skin is erythematous, swollenand slightly scaly. With healing,areas of depigmentation may occur.This should be differentiated froman ammoniacal diaper dermatitiswhere the rash is generally asymmet-ric, the intertriginous areas arespared, and satellite lesions areabsent as it is a contact dermatitis.

Figure 2.153. An infant with healed candidal dermatitisshowing symmetrical depigmentation. Note the healed satel-lite areas. Candidal (monilial) dermatitis is a commonly over-looked disorder and should be suspected whenever a diaperrash fails to respond to usual measures.

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Figure 2.154. Congenital cutaneous candidiasis in an infant at age 3 days.Potassium hydroxide (KOH) scrapings of the skin were positive for hyphae.Mucocutaneous candidiasis may present as a vesicular dermatitis in the firstweek of life. The vesicular lesions become confluent and rupture, leaving adenuded area surrounded by satellite lesions or pustules. Congenital candidi-asis may have only skin manifestations or there may be severe systemicinvolvement following the intrauterine infection.

Figure 2.155. Close-up of the skin of the same infant as in Figure 2.154. Notethe pinpoint pustulovesicular lesions. Diagnosis can be confirmed by cultureon Sabouraud’s or Nickerson’s media.

Figure 2.156. Congenital dis-seminated mucocutaneous can-didiasis in an infant at age 5 days.Note the thrush with the general-ized skin rash. These infants aregenerally well and have no sys-temic involvement. Althoughcutaneous candidiasis frequentlyoccurs in association with oralthrush, commonly the mouth isbypassed and the infection is con-fined exclusively to the diaperarea. Infants harbor Candida albi-cans in the lower intestine, and itis from this focus that infectedfeces present the primary sourcefor candidal diaper rash.

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Figure 2.157. Close-up of themouth and face in the sameinfant as in Figure 2.156. Thewhite plaques on the tongue andbuccal mucosa resemble milkcurds; they are difficult to detachfrom the mucosa and leave a rawerythematous base.

Figure 2.158. This premature infant developedcandidal infection of the nails (onychomycosis) at theage of 3 weeks. Premature infants with thrush are morelikely to develop infection of the nails from hand-mouth contact. Candidal infection almost exclusivelyaffects the fingernails, whereas tineal infection morefrequently affects toenails. In candidal infection thenail plate may be fragile and thick and may showgrayish-white spots or brown discoloration of the nailedge. As there frequently is an associated paronychiain candidal infection, the adjacent cuticle is pink,swollen, and tender (caused by secondary staphyloccal infection). (Levy, M., Moise, K.)

Figure 2.159. Candidal endophthalmitis as repre-sented by the appearance of “cotton” patches isobserved in an infant with systemic candidiasis. Theselesions are diagnostic of a systemic candidal infection.

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Figure 2.160. Candidal infectionof the back and buttocks in asmall premature infant (birth-weight 775 g) at the age of 10days. Hyphae were present bothon the skin and in the urine.

Figure 2.161. Candidal infectionof the back in a premature infantof 24 weeks gestation (birthweight680 g). The severe skin involve-ment was noted at 9 days of agewhen the condition of the infantwas stable. There was minimalhandling of the infant prior tothis. This infant developed apatent ductus arteriosus whichrequired ligation. Intravenoustherapy with amphotericin wasnot successful.

Figure 2.162. Severe dissemi-nated candidiasis in a prematureinfant (birthweight 690 g). Onthe 13th day of life he developedthe severe rash on his back.Hyphae were present in theselesions. The infant had resolutionof fungemia and skin involvementwith amphotericin therapy.

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Figure 2.163. Candidal arthritisof the right ankle in an infant whodeveloped candidal sepsis while onprolonged total parenteral nutri-tion for short bowel syndrome.

Figure 2.164. Candidal scalpinfection in an infant on periph-eral parenteral nutrition. Theabscesses were recurrent until thecloudy appearance of the infusionsolution was noted. Hyphae wereseen on examinaton of this fluid.The abscesses resolved with dis-continuation of the concentratedglucose solution.

Figure 2.165. Invasive asper-gillosis of the skin in a smallpremature infant (birthweight 785g) at the age of 7 days. Note theinvolvement of the back and theright axilla. Infant was treatedwith amphotericin.

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Figure 2.166. The same infantas in Figure 2.165 at the age of 5weeks, showing healing of thelesions.

Figure 2.167. The same infantas in Figure 2.165 and 2.166 atthe age of 10 weeks with goodhealing of the skin of the backand axilla. However, the con-tractures that were presentnecessitated plastic surgery at alater date.

Figure 2.168. Premature infant(birthweight 1000 g) with severehyaline membrane disease withgiant omphalocele. Respiratoryproblems precluded surgicalintervention. The omphalocelewas managed medically with theapplication of mercurochrome.The infant developed mercuryintoxication from excessiveabsorption of the mercuro-chrome.

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Figure 2.169. The same infant as inFigure 2.168 at the age of 11 days.Note the fungus on the omphalocele.KOH scrapings were positive forhyphae, and culture grew Aspergillusfumigatus. Aspergillosis is an uncom-mon opportunistic fungal disease.Aspergillus fumigatus is the most com-mon, but the incidence of Aspergillusniger and other species is increasing.These fungi are ubiquitous and nor-mally nonpathogenic. Primarily theyaffect debilitated individuals.

Figure 2.170. The same infant as inFigure 2.168 and 2.169 at the age of18 days showing generalized involve-ment of the skin. The skin lesions arecharacterized by erythematous papuleswhich develop into hemorrhagic bul-lae and then become violaceousplaques with central necrosis.

Figure 2.171. This small premature infant born at 23 weeks gestation (birth-weight of 580 g) had severe hyaline membrane disease and a large ductusarteriosus. She was referred at the age of 33 days, having developed aStaphylococcus epidermidis bacteremia, bilateral grade III intraventricular hem-orrhages, and bronchopulmonary dysplasia. Shortly after admission, infectionof the right forearm and hand developed which was progressive, becominggangrenous. Rhizopus infection was shown to be the etiology. She died at theage of 83 days.

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Figure 2.172. Another view of thearm and hand of the same infant asin Figure 2.171. Phycomycosis isvery rare in neonates. The mostcommon genera include Mucor,Absidia, and Rhizopus. Fungi of thisclass are frequently found in refriger-ators and are commonly known asbread molds. Phycomycetes havethe same affinity for vascular inva-sion, hemorrhage, necrosis, and sup-puration as Aspergillus. There arefew reports in the neonatal period,and the majority of these infantshave died.

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147

Abdomencreased, 2distended, 46, 111, 118

Abrasions, 16-17, 19

Abscessbreast, 91miliary, 102scalp, 89, 107xiphoid area, 92

Absidia, 145

Achromobacter, 103

Acne neonatorum, 14

Acrocyanosis, 7

Adams-Oliver syndrome, 32

Adenopathy. See Lymph nodes

Adnexal tissues, 68

Albinism, 53-54. See also Hypopigmentation

Alopecia, 30, 33, 79, 81, 137

Amastia, 30

Aminoaciduria, 79

Amniotic bands, 31-32

Amniotic fluid, 6, 101

Anasarca, 44

Androgen, maternal, 12, 14

Anemia, 18, 82, 111, 118, 126, 136

“Angel’s kiss” (macular hemangioma), 36-37

Anhidrotic ectodermal dysplasia, 79-80

Apert’s syndrome, 80

Aplasia cutis congenita, 30-32

Areola. See Nipples

Arthritiscandidal, 142gonococcal, 101syphilitic, 116

Ascites, 111, 113

Ash leaf spots, 52

Aspergillosis, 142-44

Asphyxia, 36

Astigmatism, 40

Athelia, 30

Atrioventricular block, congenital, 81

Axillary freckling, 56

Bacterial infections, perinatal, 88-117

Bacteroides, 97

Balloon cells, 125

Basal cell carcinoma, 69

Beta hemolytic streptococcal sepsis, 99

Betadine, 1

“Bilirubin” rash, 7

Black infants, 2, 7-8, 11, 55-56

Blaschko’s lines, 62, 64-66

Blistering. See Skin lesions, bullous or vesicular;Epidermolysis bullosa; Sucking blisters;Vesiculobullous eruptions

Bloch-Sulzberger syndrome, 62-64

“Blueberry muffin” skin lesions, 118-19, 127-29, 136

Bones, “abnormal” development. See Growtharrest lines, Incontinentia pigmenti,Osteochondritis, Osteomyelitis, Osteoporosis,Periostitis, Rubella, Syphilis

Boric acid, 1

Brainatrophy, 41, 68necrosis, 136-37

Breastabscessed, 91absent, 30engorgement, 27supernumerary, 29See also Nipples

Breech presentation, 19-20, 124

Bronchopulmonary dysplasia, 144

Buccal mucosa, pigmented, 56

Bullae. See Skin lesions, bullous

Bullous bleb, 31

Bullous congenital ichthyosiform erythroderma,62, 75-76, 108

Bullous impetigo, 62, 88, 108

Bullous mastocytosis, 62

Buphthalmos, 41, 130

Bursitis, 98

Café-au-lait spots, 52, 55-56

Calcificationcerebral cortex, 41

Index

148

intracranial, 122, 135-36“railroad track,” 41subcutaneous fat necrosis, 19-21

Candidal infection, 11, 94, 138-42

Capillaries, dilated, 36

Capillary hemangioma, 38-40

Capsular distention, 93

Caput succedaneum, 124

Carcinoma, basal cell, 69

Cardiac defects, 52, 63, 127, 133

Cardiovascular collapse, 125

Cartilage-hair hypoplasia, 33

Cataracts, congenital, 63, 129, 133, 136

Caucasian infants, 7, 55

“Celery stalk” appearance of bones, 122, 131-32

Cellulitis, 91-92, 98-101, 103, 105

Central nervous system, 8, 60, 68, 78, 125-26

Cephalhematoma, infected, 93

Cerebral cortex, 41, 52

Cerebrospinal fluid, 136

Cheilitis, 111

Chicken pox. See Varicella

CHILD syndrome, 78

Chorioretinitis, 112, 118-19, 133, 136

Circumcision, 98

Clostridium tetani, 106

Clutton’s joints, 116

Coagulopathy, 105, 125

Collagen, 80

“Collodion baby,” 73

Colobomata, of eyelid, iris or choroid, 68

Comedones, 14

Condylomata, 112

Congenital alopecia, 33

Congenital atrioventricular block, 81

Congenital bullous ichthyosiform erythroderma,62, 108

Congenital cataract, 129, 133, 136

Congenital cutaneous candidiasis, 139

Congenital cytomegaloviral infection, 118-22,128, 135

Congenital generalized fibromatosis, 84-85

Congenital generalized lymphangiectasia,46-47

Congenital hemidysplasia with ichthyosiformerythroderma and limb defects (CHILDsyndrome), 78

Congenital hyperthyroidism, 16

Congenital ichthyosis, 76-77

Congenital ichthyosiform erythroderma, 62, 73,75, 108

Congenital leukemia, 128

Congenital listeriosis, 101-3

Congenital pneumonia, 102

Congenital rubella, 118, 122, 127-33

Congenital scalp defect, 30-32

Congenital self-healing reticulohistocytosis,83

Congenital syphilis, 93, 108-17

Congenital toxoplasmosis, 121, 128, 135-36

Congenital varicella, 133

Congestive heart failure, 43-44

Conjunctivaepigmented, 56reddened, 77, 105

Coombs’ test, 81

Corneal clouding, 68, 130

Cornelia de Lange’s syndrome, 34

Coxsackie virus, 117

Credé’s method, 100

Crowe’s sign (axillary freckling), 56

Cutaneous candidiasis, congenital, 139

Cutaneous melanosis, 60-61

Cutis verticis gyrata, 80

Cutis marmorata telangiectatica congenita, 8, 46-47

Cystic hygroma, 50

Cysts, inclusion, 12, 28

Cytomegalovirus infection, 118-22, 128, 135

Dacryocystitis, 90

Dactylitis, 113

149

Darier’s sign, 61-62

Deafness, 54, 112, 127. See also Hearing loss

Dehydration, 74

Depigmentation, 138

Dermal erythropoiesis, 118, 128

Dermatitiscandidal/monilial, 138-39exfoliative, 97“flea-bite,” 9-11

Dermatographism, 61-62

Dermoidsnasal, 82ocular, 68

Desquamation, 2, 5-6, 75, 79, 82, 99, 108

Diaper rash, 138-39

Diarrhea, 136

Diffuse lymphangioma, 51

Diffuse neonatal hemangiomatosis, 45-46

Digits. See Fingers, Toes

Dimpling, of skin, 21-22

Disseminated intravascular coagulopathy, 105, 125

Dopa-positive melanocytes, 7

Drug withdrawal, 16-17

Dystocia, 111

Dystopia canthorum, 54-55

Dystrophic epidermolysis bullosa, 70, 72

Ears, hairy, 33

Ecchymoses, 18-20, 124-25

Eclabium, 73-74, 77

Ectasia, developmental, 47

Ectodermal dysplasia, 30, 33

Ectrodactyly-ectodermal dysplasia-clefting (EEC) syndrome, 88

Ectropion, 73-74, 77

Edema, 18, 108, 124

EEC syndrome, 80

Ehlers-Danlos syndrome, 80

Elephantiasis congenita angiomatosis/lymphangiectatica, 51

Encephalitis, 123-25

Encephalomyelitis, 133-34

Encephalotrigeminal angiomatosis, 41-42

Endophthalmitis, candidal, 140

Eosinophils, 10-11, 62, 82, 84, 136

Ephelides, 11

Epidermolysis bullosa, 62, 70-73, 75, 108

Epidermolytic hyperkeratosis, 75-76

Epilepsy, of mother, 34

Epiphyseal dislocation, 115

Epiphyseal system, 94

Epitrochlear glands, enlarged, 130

Erb’s palsy, 115

Erythema, 9-11, 75, 81-82, 92, 96

Erythematous maculopapular exanthem, 82

Erythroblastosis fetalis, 3, 114, 128

Erythroderma, 62, 73-76, 78-79, 82, 108

Escherischia coli, 91, 94, 103

Estrogen, maternal, 27

Etretinate, 77

Eucerin, 77

Extrophy of the cloacal sequence, 23

Eye disorders, 41, 122, 127, 133

Eyebrowsabsent, 79bridged, 34

Eyelashes, straight, 34

Eyelidseversion of, 73-74, 77lacrimal puncta, 54

Familial progressive hyperpigmentation, 56-57

Fasciitis, 97, 103-4

Feet, 23, 73, 108-10. See also Toes

Fetal hydantoin syndrome, 35

Fibromatosis, congenital generalized, 84-85

Fibular hypoplasia, 23

Fingershypoplastic, 78spindle-shaped, 113unseparated, 72

150

“Flea bite” dermatitis, 9-11

Forceps delivery, 17, 19, 90

Forelock, white, 54-55

Freckles, 11

Fungal infections, 81, 138-45

Funisitis, 92

Furunculosis, 88

Genitalia, lesions on, 124. See also Penis

Glaucoma, 41-42, 130

Goldenhar’s syndrome, 26

Gonococcal infections, 100-101

Graft-versus-host (GVH) disease, 82

Granuloma infantisepticum, 103

Growth arrest lines, 114-15, 132

Growth retardation, 128

Haemophilus influenzae cellulitis, 101

Hair development, 33follicle, hamartoma of, 83

pigmentation, 54tufts, 24See also Alopecia

Hairline, 34

Hallermann-Streiff syndrome, 33

Hamartomaof hair follicle, 83renal, 52

Hand-Schüller-Christian disease, 84

Handsdesquamation of, 108hyperkeratosis of, 73ulceration of, 109

Harlequin fetus, 9, 76-77

Harlequin sign, 8-9

Hashimoto-Pritzker disease, 83

Hearing loss, 118, 133. See also Deafness

Hemangiomacapillary, 38-40, 44cavernous, 42-45hemangiolymphoma, 49-50

macular, 36-38, 41multiple, 45-46papular, 45

Hematemesis, 46

Hepatitis, 125, 134

Hepatosplenomegaly, 62, 81, 111, 118, 124, 127, 136

Herlitz’s disease (junctional epidermolysisbullosa), 70, 72

Herpes, 11, 88-89, 122-26

Heterochromia iridis, 54

Hexachlorophene, 1

Higouménaki’s sign, 117

Hirsutism, 6, 33-34

Hispanic infants, 6-7, 33-34

Histamine release, 61

Histiocytosis X, 83-84

Hutchinson’s triad, 112

Hyaline membrane disease, 143-44

Hydrocephalus, 60, 135-37

Hydrops fetalis, 126-27

Hygroma colli, 50

Hyperactivity, 16-17

Hyperbilirubinemia, 7, 18, 127

Hypercalcemia, 20

Hyperkeratosis, 66, 73, 75

Hypernatremia, 74

Hyperpigmentation, 10-11, 56-57, 62, 64-66, 79, 112

Hyperpituitarism, 80

Hypersensitivity to drugs, 81

Hyperthyroidism, congenital, 16

Hypertrichosis, 6, 33-34

Hypertrophyof arm/leg, 45of epidermis, 66of shoulder, 44

Hyphae. See Fungal infections

Hypodontia, 63

Hypohidrotic ectodermal dysplasia, 79-80

Hypomelanosis of Ito, 65

151

Hypophosphatasia, 22

Hypopigmentation, 51, 65, 81, 112. See also Albinism

Hypoplasiacartilage-hair, 33fibular, 23of limbs, 133

Hypothermia, 102

Hypothyroidism, 8

Hypotrichosis, 33

Ichthyosis, 9, 62, 73-79, 108

Immunodeficiency syndrome, 82

Impetigo, 62, 88, 108

Incisions, 18

Inclusion cysts, 12

Incontinentia pigmenti, 62-64achromians, 65

Infantile cortical hyperostosis, 114

Interstitial keratitis, 112

Intracranial calcification, 122, 135-36

Intracranial extension, 82

Intracranial malformations, 68

Intrauterine sucking lesions, 14-15

Intravascular coagulopathy, 43

Irides, translucent, 52

Jaundice, 111, 118, 124, 136

Joints, swollen, 93-94

Junctional epidermolysis bullosa, 70, 72

Juvenile xanthogranulomatosis, 83

Kasabach-Merritt syndrome, 43

Keloids, 72

Keratin, 12

Keratinization, faulty, 73

Keratitis, 124

Kidney cells, viral infection, 120-21

Klebsiella, 94

Klippel-Trenaunay syndrome, 44-45

Knees, effusion in, 116

Koebner phenomenon, 71

Langerhans’ cells, 83-84

Larsen’s syndrome, 35

Lentigines, 10-11

Leprechaunism, 34

Lesions. See Ocular lesions, Osseous lesions,Retinal lesions, Skin lesions

Letterer-Siwe disease, 84

Leukemia, congenital, 128

Leukoderma, 51, 54

Leukopenia, 81, 136

Linea nigra, 2-3

Linear nevus sebaceus syndrome, 68

Lips, eversion of, 73-74, 77, 79

Listeria monocytogenes, 101-3

Listerial meningitis, 102

Listeriosis, congenital, 101-3

Lungs, 113, 120

Lupus erythematosus, neonatal, 81

Lymph nodes, enlarged, 130

Lymphangioma, 48-51

Lymphedema, 47-48

Macroglossia, 49

Macular chorioretinitis, 120

Magnetic resonance imaging (MRI), 25

Mastitis, 27-28, 91

Mastocytosis, 61-62

Maxillary alveolar processes, hypoplastic, 80

McCune-Albright polyostotic dysplasia, 55

Meconium staining, 3-5, 101

Melanoblasts, 60

Melanocytes, 7, 52, 54, 57, 59

Melanoma, 58-61

Melanosis, transient neonatal pustular, 10-11

Melanosomes, 52, 54

152

Meningitis, 102, 104

Meningoencephalitis, 117, 125, 127

Mental retardation, 52, 63, 65, 118

Metaphyseal system, 94

Microcephaly, 63, 118, 121, 135

Microphthalmia, 129, 136

Microsporonaudouinii, 137canis, 137

Midline defects, 24-27

Midline demarcation, 78

Milia, 12

Miliaria crystallina, 13

Miliaria rubra, 13

Milk line, 28-29

Milroy’s disease, 48

Molars, Moon’s, 112

Mongolian spots, 7-8, 20, 42

Monilial diaper dermatitis, 138-39

Moon’s molars, 112

Mucor, 145

Mulberry molars, 112

Nailsabrasions caused by, 16absent, 77-78aplasia, 35brittle, 79broad, 35candidal infection of, 140dystrophy of, 72hypoplastic, 35, 77-78nail-patella syndrome, 35triangular, 34

Nasal dermoid, 82

Nasal mucosa, ulcerated, 111

Neck, skin folds, 27

Necrotizing fasciitis, 97

Neisseria, 90, 100

Neonatal lupus erythematosus, 81

Neural axis/neural tube defect, 24, 60

Neurocutaneous melanosis sequence, 60-61

Neurofibromatosis, 11, 55-56, 83Neurologic sequelae, 118Neurosyphilis, 112Neutrophils, 11Nevocytes, 57Nevusanemicus, 38blue, 8compound, 59epidermal, 66-69faun-tail, 60flammeus (port wine stain), 36, 41-42, 44“garment,” 59hairy, 58, 60halo, 57hamartomous, benign, 70junctional, 57-59linear nevus sebaceus, 68pigmented, 57-60, 67retinal, 52sebaceus of Jadassohn, 66, 68-70“stork-bite,” 37strawberry, 38-40telangiectatic, 37-38unius lateris, 68vascular, 36verrucous, 62, 64, 66-67, 69verrucosus, 67See also Skin lesions

Nikolsky’s sign, 70-71, 79, 96

Nipplesabsent (athelia), 30cysts on, 28hypoplastic, 30pigmentation of areola, 2spacing of, 28, 30supernumerary, 29-30

Non-bullous ichthyosiform erythroderma, 73, 79

Noonan’s syndrome, 27, 30

Nosocomial infection, 89, 92

Nostrils, anteverted, 34Nuchal cord, 18, 36Nystagmus, 52

Ocular dermoids, 68

Omphalitis, 92

Omphalocele, 106, 143-44

153

Onychomycosis, 140

Ophthalmic abnormalities, 68

Opisthotonos, 130

Optic atrophy, 41

Oral-facial-digital syndrome type 1, 12

Oriental infants, 7

Osseous lesion, 115

Ossification, delayed, 115

Osteochondritis, 113, 115

Osteomyelitis, 93-94, 116

Osteoporosis, 114

“Owl’s eye” appearance of kidney cells, 120-21

Pachydermomyositis, 80

Papules, 12

Paracolobactrum, 103

Parasitic infections, 135-38

Parenteral nutrition, 142

Paronychia, 140

Parotitis, 90

Parrot’s atrophy of newborn, 115

Parvovirus B19 infection, 126-27

Patent ductus arteriosus, 127, 141, 144

“Peeling skin” syndrome, 79

Pemphigus neonatorum (bullous impetigo), 88

Penis, inflamed, 98

Periostitis, 114-17

Peripheral vasoconstriction, 7

Periventricular calcification, 121-22

Petechiae, 36, 123, 125

Phacomatosis pigmentovascularis, 42

Philtrum, absent, 34

Photophobia, 52

Phototherapy, 7

Phycomycosis. See Fungal infections

Physiotherapy, 21

Piebaldism, 54

Pigmentation, 2-3, 7-8, 11, 42, 51-67. See also Albinism, Hyperpigmentation, Hypopigmentation, Nevus

Pilonidal sinus, 22, 25

Pilosebaceous glands, 12

Platelet trapping, 43

Pneumonia, 102, 113, 125, 127, 134

Poland’s anomaly, 30

Poliosis circumscripta, 54

Port wine stain, 41, 44

Prenatal edema, 27

Prickly heat, 13

Progeria Hallermann-Streiff syndrome, 33

Proteus mirabilis, 94, 97

Protozoal infections, 135-38

Proximal tibial metaphysis, 116

Pseudomonas aeruginosa, 104-6

Pseudoparalysis, 115

Purpura, 118, 123, 127

Purpura fulminans, 105

Pustular melanosis, 11, 88

Pyoderma, 88

Rash“bilirubin,” 7diaper, 138-39generalized, 95See also Skin lesions, Syphilis, Urticaria

Renal hamartoma, 52

Reticulation of skin, 46

Reticulohistocytosis, congenital self-healing(Hashimoto-Pritzker disease), 83

Retinadysplasia, 63lesions, 52

Rhabdomyomas (cardiac tumours), 52

Rhagades, 111

Rheumatoid disease, 81

Rhinitis, 111

Rhizopus infection, 144-45

Ribs, extra, 63

154

Rickets, 114

Ringworm, 137

Risus sardonicus, 106-7

Ritter’s disease, 96-97

Rubella, congenital, 118, 122, 127-33

Rubenstein-Taybi syndrome, 35

Russell-Silver syndrome, 55

Saber shins, 116

Safflower oil, 1

Salmon patches, 36-37

Salmonella, 94, 103

Scabies, 137-38

Scalded skin syndrome, 96-97

Scaling disorders, 73-79

Scalp defects, 30-32, 95abscesses on, 89, 107candidal infection, 142cystic swelling, 40epidermal nevus, 66erythematic, 81furrowed, 80lesions on, 68, 124scaling, 137

Scarlatina, 95, 99

Sclerema neonatorum, 19

Sebaceous gland hyperplasia, 12

Sebaceous glands, 3, 12, 68

Seizures, 41-42, 52, 60, 63, 65

Sepsis, 8

Septicemia, neonatal, 93

Shagreen patches, 52

“Sheet burns,” 16

Shingles. See Varicella

Shoulder, asymmetric hypertrophy, 44

Skeletal abnormalities, 42

Skindimpling, 21-22erosion, 67folds, 24, 27peeling, 79reticulation, 46scalded skin syndrome, 96-97

tags, 24, 26-27ulceration, 23webbing, 27

Skin lesions, 2-21, 81, 101-5, 128annular, 103, 112“blueberry muffin,” 118-19, 127-29, 136bullous, 62-63, 70-73, 75, 88, 96, 108-10, 144cicatricial, 133circinate, 108, 110-12ecchymotic, 19-20edematous, 81erythematous, 9-10, 81, 123gangrenous, 106hamartomous, 69hemorrhagic, 105, 109-10, 144herpetic, 10, 122-26hyperpigmented, 64impetiginous, 88infiltrative, 84intrauterine sucking, 14-15macular, 38maculopapular, 7, 9-10, 62, 102, 110,

117, 136midline, 24necrotic, 103-4nodular, 61papular, 57papulovesicular, 83ringworm, 137scabetic, 137-38syphilitic, 108-10vascular, 119vesicular, 10, 62-64, 102, 105, 108, 124,

137-38vesiculobullous, 108-9vesiculopustular, 11violaceous, 103warty, 67-68, 112yellow, 83zosteriform, 123See also Cysts, Hemangioma, Nevus, Rash

Skull abnormalities, 131

Spinal tap, 20, 25

Splenomegaly, 111

Staphylococcal infections, 11, 88-98, 100, 125,140, 144dacryocystitis, 90funisitis, 92furunculosis, 88omphalitis, 92osteomyelitis, 93-95

155

parotitis, 90pyoderma, 88scalded skin syndrome, 96-97

Staphylococcus aureus, 91-98

Staphylococcus epidermidis, 144

Stratum corneum, abnormalities of, 73-79

Stratum granulosum, 79

Strawberry nevus, 38-40

Streptococcal infection, 88, 90, 98-100

Sturge-Weber syndrome, 41-42

Subcutaneous fat necrosis, 19-21

Sucking blisters, 14-15

Sudamina, 13

Suffusion of face, 18

Sweat glands, 13absent, 79

Synophrys, 34, 54

Syphilis, congenital, 93, 108-17

Syphilitic pemphigus, 105, 108, 110

Tail, 26

Teethabsent, 80effects of syphilis, 112hypodontia, 63

Tetanus, neonatal, 106-7

Thrombocytopenia, 43, 81, 98, 105, 111, 118,127, 136

Thrush, 139-40

Thumbs, digitalization of, 35

Tibia, bowed, 116

Tinea capitis, 137

Toesbroad, 35hypoplastic, 77-78unseparated, 72

Toxic epidermal necrolysis, 75, 96-97

Toxoplasma gondii, 135

Toxoplasmosis, congenital, 121, 128, 135-36

Transient bullous dermolysis, 73

Transphyseal vessels, 94

Trichophyton tonsurans, 137

Trigeminal nerve, 41

Trismus, 106

Trisomy 13, 31

Tryptophan, low, 79

Tuberous sclerosis, 51-52, 55, 80

Tumor, cardiac, 52

Turner’s syndrome, 27, 30, 47-48

Tyrosinase-positive albinism, 54

Umbilical cord, infection of (funisitis), 92

Urine analysis, 121

Urticarianeonatorum, 9pigmentosa, 61-62

Vancomycin, 92

Varicella, 32, 133

Vascular disorders, 36-51

Vasculitis, 118-19

Vasoconstriction, peripheral, 7

Vasomotor instability, 8

Ventricles, dilated, 122

Vernix caseosa, 1-5

Vesiculobullous eruptions, 70-73

Viral infections, 117-34

Vitiligo, 51-52

Waardenburg’s syndrome, 34, 54-55

Webbing of skin, 27

Wegner’s sign, 114

Wimberger’s sign, 116

Witch’s milk, 27

Xanthogranulomatosis, juvenile, 83

Xanthoma, 61

Zosteriform eruption, 123

Atlas of the Newborn [Vol 4] - A. Rudolph (BC Decker, 1997) WW

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Transcript of Atlas of the Newborn [Vol 4] - A. Rudolph (BC Decker, 1997) WW