Childhood Psoriasis: A Clinical Review of 1262 Cases

Childhood Psoriasis: A Clinical Review of1262 Cases

Anne Morris, M.B., B.S.,* Maureen Rogers, M.B., B.S., F.A.C.D.,†Gayle Fischer, B.Arch., M.B., B.S., F.A.C.D.,† and

Katrina Williams, M.B., B.S., M.Sc., F.R.A.C.P., F.A.F.P.H.M.*

Departments of *Clinical Epidemiology and †Dermatology, Royal Alexandra Hospital for Children,Westmead, New South Wales, Australia

Abstract: Our aim was to describe the types of psoriasis seen in a largeseries of patients presenting to a tertiary referral pediatric dermatologydepartment using a classification system combining conventional termi-nology and additional categories based on the site and characteristics ofthe rash. A total of 1262 patients seen consecutively in the dermatologydepartment of the Royal Alexandra Hospital for Children, Sydney, Aus-tralia, between 1981 and 1995 are described and classified according tothe pattern of psoriasis at the time of presentation. Additional informationrecorded included family history, facial involvement, and history of a pso-riatic type of diaper rash in infancy. The ages of the children ranged from1 month to 15 years. There was an equal gender distribution and a highrate of positive family history at 71%. Twenty-six percent of children hada history of a psoriatic diaper rash and facial involvement occurred in 38%of children. Plaque psoriasis was the most common type overall, affecting430 patients (34%). Three hundred forty-five children were less than 2years of age, and this is the largest series of children with psoriasis in thisage group presented to date. An entity defined by us as psoriatic diaperrash with dissemination was the most common type of psoriasis in theless than 2-year age group, affecting 155 (45%) patients. This large seriesoffers information on the manifestations of psoriasis in childhood, but isparticularly useful in examining the previously less well-described infantage group. The classification used is proposed as a practical way todescribe psoriasis in children, particularly with respect to future descrip-tive studies.

Psoriasis in childhood is a disease of many forms thatmay change over time. Its true incidence is unknown.Also unknown is the natural history of childhood onsetpsoriasis. What is known is that the frequencies of sometypes or patterns of psoriasis differ between adults and

children, with several adult types rarely described in chil-dren. In the study of Farber and Nall (1), 35% of adultsufferers were reported to have had onset of disease be-fore the age of 20 years. Nyfors and Lemholt (2), in areview of the literature from Scandinavia, reported that

Address correspondence to Maureen Rogers, MB, Suite 19, Chil-dren’s Hospital Medical Centre, Hainsworth Street, Westmead, NSW2145, Australia.

Pediatric Dermatology Vol. 18 No. 3 188–198, 2001

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45% of adult psoriatics from that region had onset beforeage 16 years. Incidence estimates have been hamperedby a lack of clarity about defining what is and what is notchildhood psoriasis. Psoriatic diaper rash with dissemi-nation is the most outstanding example of this and muchdebate has occurred over the terminology and validity ofits inclusion within the classification of psoriasis. Thiscase series presents the experience of a tertiary pediatricdermatology service over 14 years. Children with diaperdistribution disease are included to address the issue ofdefinition in an attempt to clarify the terminology.

METHODS

Between 1981 and 1995 information was collected aboutall children diagnosed with psoriasis by two pediatricdermatologists associated with the Royal AlexandraHospital for Children, Sydney, Australia. Informationwas recorded for all children seen consecutively in eitherthe pediatric dermatology clinic or private practice.

Classification of the type of psoriasis was made usingconventional terms as used previously in other case se-ries. In addition, more specific groups based on site andcharacteristics of the rash were used (Appendix) An ad-ditional category was included for children with featuresintermediate between eczema and psoriasis. This classi-fication is more detailed in order to describe the diverserange of manifestations of childhood psoriasis.

The type of psoriasis recorded was based on findingsat the time of presentation to the dermatologist. Infor-mation about age, gender, family history in a first-degreerelative, involvement of the face, and history of a psori-atic diaper rash was recorded. Descriptive analysis wasperformed using SPSS for Windows, version 6.0 (SPSSInc., Chicago, IL).

RESULTS

A total of 1262 children were seen during the studyperiod. Ages ranged from 1 month to 15 years. Twohundred four children (16%) were less than 1 year of ageand 345 (27%) were less than 2 years of age (Fig. 1).

The gender distribution was approximately equiva-lent, with 588 (47%) boys and 674 (53%) girls. Thescalp-only group had the most marked female predomi-nance. There was no difference in gender distributionaccording to age. The overall frequency of the differenttypes of psoriasis seen is shown in Table 1.

Plaque psoriasis was the most frequent type seen, ac-counting for 430 cases (34%). Facial rash was the solemanifestation in 54 children (4%). However, 480 chil-dren (38%) had facial involvement as part of their rash,including just over 40% of children each with plaque andguttate psoriasis.

One hundred sixty-one children (13%) presented withpsoriatic diaper rash with dissemination and 45 (4%)with localized psoriatic diaper rash. When patients withdiaper area presentations were excluded, 277 (26%) ofthe remaining 1056 children had a positive history of apsoriatic diaper rash. Seven hundred fifteen (68%) had anegative history and in 63 (6%) the information wasunavailable or the parent was unsure.

In the group less than 2 years of age, the most com-mon diagnosis was psoriatic diaper rash with dissemina-tion (155 patients). Excluding cases with psoriatic diaperrash with dissemination and localized psoriatic diaperrash, the rank order of the most common two types(plaque and scalp alone) was the same in the two agesubgroups (Table 2). However, anogenital psoriasis oc-curred in four times as many children more than 2 yearsof age as less than 2 years of age.

Figure 1. Age distribution of patients.

TABLE 1. Type of Psoriasis

Type Number Percentage

Plaque 430 34.1Psoriatic diaper rash with

dissemination 161 12.7Scalp 146 11.5Anogenital 112 8.9Guttate 81 6.4Acropustulosis 60 4.7Eczema-psoriasis overlap 55 4.3Face plaque only 54 4.3Palm and sole 50 3.9Localized psoriatic diaper rash 45 3.6Annular/nonpustular 26 2.1Follicular/micropapular 24 2.1Pustular 8 0.6Nail 8 0.6Erythrodermic 1 0.1Linear 1 0.1Total 1262 100

Morris et al: Childhood Psoriasis189

Family History

Overall, information about family history was availablein 773 patients (61%). A positive family history of pso-riasis in a first-degree relative was present in 71% ofthose for whom such information was available. Whenpatients with the psoriatic diaper rash presentations wereexcluded, the rate of positive family history was similar,at 73%. In the 108 patients with psoriatic diaper rashwith dissemination where family history information wasavailable, 68 (63%) had a positive family history, and inthe 31 patients with localized psoriatic diaper rash, 22(71%) had a positive history.

DISCUSSION

This is the largest case series of children with psoriasisreported to date. An important advantage of this series isthe large number of consecutive patients on which thedata have been compiled. The information collected re-flects the nature of this condition seen in a tertiary re-ferral children’s inpatient and outpatient facility over 14years. Consequently some of the rarer forms of child-hood psoriasis are described in numbers greater than pre-viously reported. In addition, specific aspects have beenprospectively recorded, including facial involvement andthe more controversial history of a psoriatic diaper rash.

Methodology

The series differs somewhat from others in that we reportthe findings at first presentation to the dermatology ser-vice rather than strictly at the onset of symptoms. Wesuggest, however, that in many cases the two would havecoincided. The study design does not allow considerationof etiologic factors or management outcomes.

Gender

Previous reports have observed a female preponderanceapproaching 2:1 (2–5). This study, with an equal genderdistribution, is similar to a report from India (6). It isunclear why this difference from other studies has oc-curred, but it may reflect different referral patterns basedon gender issues for the various study locations. On ex-amining the gender distribution with respect to type inthose with sufficient numbers, in the scalp-only groupalone there was a significant female preponderance. Thegender distribution was also equal in those less than andmore than 2 years of age.

Family History

A positive family history of psoriasis has been reportedin other series ranging from 9.8% to 73% (1–3,6). In ourstudy a positive family history referred to a diagnosis ofpsoriasis in a first-degree relative only. Acknowledgingthat psoriasis can run a fluctuating course in adulthood,recall of the presence of disease in another family mem-ber may be difficult. The positive family history rate of71% in this series for children with an available familyhistory may be a biased overestimate, with affected fami-lies being more likely to have confident recall aboutinvolvement of other family members. The minimumestimate for this study is a 43% positive family history,if all cases are considered in the denominator. The issueof familial tendency is still a major area of research andnot one considered specifically in this study.

Age Distribution of Patients

In comparison with other series of childhood psoriasis,the age distribution is notably different in that we had a

TABLE 2. Type of Psoriasis According to Age Group, Excluding Diaper Area Types andEczema/Psoriasis Overlap

Type

<2 Years Old >2 Years Old

Number Percentage Number Percentage

Plaque 70 53 360 41.4Guttate 8 6.1 73 8.4Anogenital 4 3 108 12.4Scalp 16 12.1 130 15Palm and sole 5 3.8 45 5.2Acropustulosis 11 8.3 49 5.7Pustular 2 1.5 6 0.7Annular nonpustular 5 3.8 21 2.4Nail 1 0.8 7 0.8Follicular/micropapular 3 2.3 21 2.4Erythrodermic 0 0 1 0.1Linear 0 0 1 0.1Face plaque only 7 5.3 47 5.4Total 132 100 869 100

190 Pediatric Dermatology Vol. 18 No. 3 May/June 2001

larger proportion of infants and young children, with27% less than 2 years of age. Nyfors and Lemholt (2), ina survey of 245 children in Denmark in 1975, reported6% with the onset of symptoms at less than 2 years of ageand an average age of onset of approximately 8 years.Other series report an onset in infancy (less than 12months) of 6.25% (6) and 8% (5). Our study includes thelargest number of children less than 2 years of age withpsoriasis. Two factors may have produced this apparentincrease in the proportion of children less than 2 years ofage. First, the inclusion of the two forms of diaper areainvolvement, as described in the method, is likely to haveincreased the proportion of infants. However, if the 198children less than 2 years of age who presented withdiaper distribution rashes are excluded, the proportion ofchildren less than 2 years of age (14%) is still higher thanin other series.

The second possible cause of an increased proportionof young children is local referral patterns. Our depart-ment is part of a large pediatric hospital, so it is possiblethat younger children are more likely to be referred hereand that teenage patients are referred to a combined adultand pediatric service which may be more similar to thesource of patients in other published series. The impactof referral practice cannot be quantified by this study.

Age and Pattern of Presentation

Considering the subgroup of 345 children less than 2years of age, the most common psoriatic type was diaperrash with dissemination. However, when the diaper dis-tribution rashes were excluded, the rank order of thetypes of psoriasis in the two age subgroups (i.e., less thanand more than 2 years of age) was almost identical. Thus

plaque and scalp alone are the most common types, re-gardless of age, with the only major difference being thegreater proportion of older children having the anogeni-tal distribution type (12.3%) compared with those lessthan 2 years of age (3%). In addition, all other types ofpsoriasis, according to our classification, except forerythrodermic and linear, were found in the less than2-years age group, illustrating the diversity of manifes-tations of the condition and the existence of the raretypes, even in young infants.

Plaque Psoriasis

In children, plaques are often smaller and the scale finerand softer than in adults. In dark-skinned children thescale may be so subtle that the condition presents ashypopigmentation and the scale becomes obvious onlywhen the lesions are scratched. Considering the overallfrequencies of the types of psoriasis, we found plaquepsoriasis to be the most common form, as in other series.However, in our series plaque psoriasis occurred in 34%of children compared with rates of 69% (6) and 84% (5)in other series. The inclusion of a large number of diaperarea cases, which may not have been included in otherseries, would have lowered this figure. Also it is possiblethat some cases classified here as anogenital, face plaqueonly, or scalp only would have been considered as plaqueby other authors. If the diaper area cases are excluded,plaque psoriasis accounts for 40%.

Guttate Psoriasis

Another marked difference between our series and othersis the number of cases of guttate psoriasis (Fig. 2), at6.4% of presentations. In previous reports the figures

Figure 2. Guttate psoriasis with smallscaly papules in a widespread distri-bution.


range from 11% (5) to 44% (2). The association of theguttate form with recent streptococcal infection, particu-larly of the throat or perianal area, is well described, sothe incidence of guttate psoriasis in children must tosome extent reflect the nature of streptococcal disease inthe community. Again, the fact that our series had apreponderance of younger patients, with relatively fewearly adolescents, may have had an effect on the fre-quency of guttate psoriasis.

Rarer Presentations

Many types of psoriasis seen in adults are describedrarely in children and our series was similar to otherpediatric series with respect to the pustular, erythroder-mic, linear (Fig. 3), and follicular forms (Fig. 4). Thechild whose disease was classified as linear psoriasispresented with a several-month history of a Blaschkolines distributed rash on her arm and trunk (Fig. 3). Thelesions were clinically and histopathologically typical ofpsoriasis and improved considerably with the applicationof a tar preparation. We regard this as a case of nevoidpsoriasis as described by Atherton et al (7). Palmoplantarpsoriasis occurred in 4% in our series, which is similar toother pediatric studies (5,6), but acropustulosis (Fig. 5)occurred at a higher than expected rate of 4.7%. Thispresentation is usually described in older female patientsbut has been noted to occur rarely in children (8). It wasinteresting to find that 68% of cases of acropustulosis inour series occurred in children less than 5 years of age.

Facial Involvement

Facial involvement (Fig. 6) has been reported as occur-ring more commonly in children than in adults (9). Pre-vious pediatric series quote rates of facial involvement at18% (3), 43% (6), and 46% (2). Our result was interme-diate at 38%. The lesions may be subtle or florid. Theyare characteristically more clearly demarcated than ec-

zematous patches, are less itchy, and quite often have anannular configuration. A common site is under the eyes.They may be the only manifestation of psoriasis, as in4% of children in this series, or occur as part of a moregeneralized rash. Facial involvement is one of the char-acteristic features of psoriatic diaper rash with dissemi-nation as defined in this series, such that 98% of childrenwe classified as such had facial lesions. Thus differencesin quoted rates in other series may be affected by theproportion of younger children included in the studies.The face was involved in slightly more than 40% ofchildren with plaque and guttate types.

Psoriatic Diaper Rashes

In this series we describe two rashes in the diaper dis-tribution on the basis of their distinctive appearances as

Figure 3. Linear psoriasis. Blas-chko distributed acquired psori-atic lesions, which respondedwell to tar therapy.

Figure 4. Micropapular psoriasis. Micropapular keratoticlesions, with alopecia suggesting follicular involvement.


described in the Appendix. We define these as localizedpsoriatic diaper rash (Fig. 7) and psoriatic diaper rashwith dissemination (Figs. 8 and 9). We propose that bothmay be psoriasis or precursors to psoriasis in some chil-dren. The two major issues that require clarification arethe validity of their inclusion within the spectrum ofpsoriasis and the use of varying terminology for the sameor similar conditions. Examples of terminology used pre-viously and descriptions of the rashes to which they havebeen applied (10–16) are outlined in Table 3. Familyhistory may be an important supportive factor for includ-ing these entities in the classification of psoriasis, par-ticularly as the genetic basis of psoriasis becomes betterunderstood. In patients with localized psoriatic diaper

rash where family history information was available,71% had a positive history, and in those with psoriaticdiaper rash with dissemination, 63% had a positive fam-ily history. These figures were close to the positive fam-ily history rate of 71% for the whole group of 773 pa-tients in whom family history information was available.

In this series psoriatic diaper rash with disseminationwas the second most common presentation overall andthe diagnosis in 45% of the children less than 2 years ofage. Thus it was the most significant form of psoriasis ininfancy with respect to frequency. Confirmation ofwhether psoriatic diaper rash with dissemination is trulya form of psoriasis is difficult because in routine cases itis unusual to perform skin biopsies in these infants, mak-

Figure 5. Psoriatic acropustulosiswith glazed erythema of the finger-tips with some pus formation andsevere nail dystrophy.

Figure 6. Psoriasis localized to theface with clearly demarcated lesionswith periorbital involvement.


ing the diagnosis a clinical one only. However, manypatients who initially presented with psoriatic diaper rashwith dissemination have been seen subsequently in ourdepartment with other forms of psoriasis. We are cur-rently undertaking a follow-up study of the cohort in thisdatabase with psoriatic diaper rash with dissemination,which will involve 161 children with a follow-up periodof up to 18 years.

The rash we have classified as localized psoriatic dia-per rash has a very characteristic appearance. Althoughrarely referred to in the literature specifically, we in-cluded it in our data both as a mode of presentation andpart of the past history to quantify the previously formedimpression of a history of unusually severe diaper rash insome children who later develop other forms of psoriasis.Some children may only ever have this characteristicwell-demarcated, bright red rash in the diaper area, whileothers have this rash as an apparent first stage, laterprogressing, often explosively, to psoriatic diaper rashwith dissemination. Psoriatic diaper rash is brighter red,better demarcated, and often shinier than seborrheic der-matitis and lacks the yellow scale that may be presentwith the latter.

In patients presenting with other types of psoriasis,slightly more than 20% had a history of psoriatic diaperrash. This history occurred in most of the types in theseries and did not appear to be related to the later devel-

opment of any particular type. The data on history ofpsoriatic diaper rash are incomplete (7% unknown) andpossibly subject to recall bias, with the more severe dia-per rashes perhaps more likely to be remembered. Be-cause of the wide age range from infancy to 15 years,there was the possibility that parents of the older childrenmay not have been able to remember the presence of asevere diaper rash as readily as those with younger chil-dren. In fact, there was no difference in recall rates be-tween age groups.

Eczema-Psoriasis Overlap

Fifty-five patients were included who presented withrashes that showed features of both eczema and psoriasis(Fig. 10). They either had an admixture of eczematousand psoriatic lesions or had lesions which were interme-diate in morphology between these two conditions. Inalmost all cases there was a past history of a typicaldiscoid eczema and most patients had a family history ofboth atopic disease and psoriasis.

Classification

Comparisons between our series and others such as thismay be made more difficult because of the varying use ofterminology as in the diaper distribution rashes but also

Figure 7. Localized psoriatic dia-per rash with well-demarcated,brightly erythematous rash, cover-ing most of the diaper area.


in well-accepted forms of psoriasis. For example, someclassifications in this series are by major diagnosticgroup based on appearance (e.g., plaque) and othersbased on site of involvement (e.g., scalp only). In par-ticular we feel the value in further classifying localizedplaque-type psoriasis in this way based on site of occur-rence may be that, with greater understanding of thepatterns of psoriasis in children, the milder or moresubtle presentations will be recognized earlier andtreated accordingly. Ideally a common classification sys-tem should be accepted that is useful both clinically andfor the future epidemiologic studies required to under-stand the natural history and response to treatment ofchildhood onset psoriasis.

CONCLUSION

In conclusion, this case series of children examines themanifestations of psoriasis across all age groups, but inparticular offers the opportunity to compare the modes ofpresentation in infants with those of older children. Pso-riatic diaper rash with dissemination aside, plaque and

scalp psoriasis were the most common types in bothgroups, with other rare types also occurring in both. Themajor differences between this and previously describedseries, besides the larger number of infants, include theequal gender distribution, lower incidence of guttate pso-riasis, and the inclusion of the two types of psoriaticdiaper rash within the classification. The high rates offamily history of psoriasis in children with diaper distri-bution rashes and the history of psoriatic diaper rashes insome older children later presenting with psoriasis areinteresting clinical observations and, we believe, validatetheir inclusion in the database.

APPENDIX: DEFINITION OF TERMS

Plaque—Well-demarcated, small to large lesions, usu-ally symmetrical. Includes flexural areas. Varying de-gree of silver-white scale

Figure 8. Psoriatic diaper rash with disseminated lesionson the trunk and well-demarcated diaper rash.

Figure 9. Psoriatic diaper rash with disseminated lesionsin widespread distribution with well-demarcated diaperrash still obvious.


Guttate—Multiple small (up to 0.5 cm) eruptive roundlesions on the trunk, limbs, and face (Fig. 2).

Localized to the scalp—Disease confined to the scalp.Discrete plaques or diffuse scale.

Localized to the anogenital area—Lesions confined tothe anogenital or groin area (Fig. 11) and are usuallywell demarcated and often glazed. For example: pso-riatic vulvitis, psoriatic balanitis.

Localized to the face—Disease confined to the face(Fig. 6)

Palm and sole—Glazed nonscaly erythema or diffuse orpatchy scaling and fissuring, often clearly demarcatedat the edges of the palms and soles (Fig. 12).

Localized to the nails—Disease confined to the nails.Annular/nonpustular—Small or large nonpustular annu-

lar lesions (Fig. 13).Follicular/micropapular—Tiny keratotic papules often

grouped into plaques especially in dark skin. Theremay some alopecia, suggesting follicular involvement(Fig. 4).

Linear—Blaschko distributed lesions. See text descrip-tion (Fig. 3).

Erythrodermic—Diffuse erythema with scale in a personknown to have psoriasis.

Disseminated pustular—Von Zumbusch generalizedpustular psoriasis, widespread plaques studded withpustules (Fig. 14) or extensive annular pustular pso-riasis.

Acropustulosis—Glazed erythema with fissuring of oneor more fingertips, often with some pustular lesions;significant nail dystrophy (Fig. 5).

Localized psoriatic diaper rash—Well-demarcated, con-fluent, bright red rash confined to the diaper area,often with a glazed appearance (Fig. 7).

Psoriatic diaper rash with dissemination—Previously re-ferred to as psoriasiform diaper dermatitis or psoria-

TABLE 3. Distribution Rash Terminology

Psoriasiform diaper dermatitisAlso known as dermatitis psoriasiformis Jadassohn

(Danish literature).Psoriasiform, confluent, arcuate, sharply demarcated, dark

red papules in the diaper area, usually with satellitepsoriasiform papules. Often spread to the trunk, limbs,scalp, and face (10–12).

Infantile seborrheic dermatitisSharply defined, round or oval red plaques in the diaper

area, which may coalesce.There may be spread to the trunk, neck, scalp, and face

(13).Psoriasiform seborrheic dermatitis

Plaques involving scalp and diaper area with laterinvolvement of body folds, trunk, and limbs. Scalesdrier and larger than true seborrheic dermatitis (14).

Diaper dermatitis with psoriasiform id eruptionInvolves intertriginous sites in the groin; widespread

(explosive) papulosquamous lesions resemblingpsoriasis (15).

Psoriasiform diaper dermatitis with idScaly, erythematous patches in the diaper region initially,

followed by explosive papulosquamous eruption on theface and trunk. The lesions are well-demarcated,psoriasiform plaques (16).

Figure 10. Eczema/psoriasis overlap. Scaly erythema-tous patches with features intermediate between eczemaand psoriasis. The child previously had typical discoid ec-zema in a similar distribution.

Figure 11. Psoriasis in the genital area of a male childwith well-demarcated glazed erythematous lesions.


siform seborrheic dermatitis. This starts with a well-demarcated erythematous rash localized to the diaperarea. A few scattered lesions develop on the trunk and

then there is subsequent, and usually explosive,spread to the face, neck, trunk, and finally the limbs(Figs. 8 and 9).

Eczema/psoriasis overlap—Scaly erythematous patches

Figure 12. Palmar psoriasis welldemarcated at the wrist fold.

Figure 13. Annular psoriasis with widespread eruption ofannular nonpustular lesions.

Figure 14. Pustular psoriasis with widespread erythema-tous patches studded with pustules.


with features intermediate between eczema and pso-riasis, often with a history of discoid eczema in in-fancy (see text description) (Fig. 10).

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Childhood Psoriasis: A Clinical Review of 1262 Cases

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