Post on 02-Apr-2015
Pruritis and Pruritis and Neurocutaneous Neurocutaneous
DermatosesDermatoses
KCOM/Texas Dermatology KCOM/Texas Dermatology Residency ConsortiumResidency Consortium
July 2003July 2003
PruritisPruritis
Fine unmyelinated C fibersFine unmyelinated C fibers Also control touch, temperature and Also control touch, temperature and
painpain Subepidermal to lateral Subepidermal to lateral
spinothalamic tract spinothalamic tract Spinothalamic tract to thalamusSpinothalamic tract to thalamus Thalamus to sensory cortexThalamus to sensory cortex
Central Itch PerceptionCentral Itch Perception
Sedation effect may decrease Sedation effect may decrease “central” itch perception regarding “central” itch perception regarding antihistamines like Atarax.antihistamines like Atarax.
Histamine is a “peripheral” mediator Histamine is a “peripheral” mediator of itch perceptionof itch perception
Naloxone is a “central” opiod Naloxone is a “central” opiod antagonist for patients with pruritic antagonist for patients with pruritic cholestasis.cholestasis.
Mediators of PruritisMediators of Pruritis
Histamine, Kinins, ProteasesHistamine, Kinins, Proteases Prostaglandin E lowers threshold for Prostaglandin E lowers threshold for
histamine induced pruritishistamine induced pruritis Enkephalins, pentapeptides which Enkephalins, pentapeptides which
bind to opiate receptors in the brain bind to opiate receptors in the brain modulate pain and itching centrally.modulate pain and itching centrally.
Interleukins implicated in AD.Interleukins implicated in AD.
Substance PSubstance P
11 amino acid peptide implicated as 11 amino acid peptide implicated as causing itching in some disorders.causing itching in some disorders.
Depletes cutaneous nociceptor nerve Depletes cutaneous nociceptor nerve endings of Substance P after endings of Substance P after repeated topical application.repeated topical application.
5-hydroxytryptamine (5-HT)5-hydroxytryptamine (5-HT)
Regulates 5-HT receptors, may be Regulates 5-HT receptors, may be therapeutictherapeutic
Ondansetron (Zofran) anti-emetic Ondansetron (Zofran) anti-emetic blocks 5-HT.blocks 5-HT.
Therapeutic in cholestatic pruritisTherapeutic in cholestatic pruritis
Variations in Intensity of Variations in Intensity of itch…itch…
Psychological traumaPsychological trauma StressStress Absence of distractionsAbsence of distractions Anxiety, fearAnxiety, fear Anatomic regions very susceptible to Anatomic regions very susceptible to
pruritis: ear canals, eyelids, nostrils, pruritis: ear canals, eyelids, nostrils, perianal, genital areas.perianal, genital areas.
TreatmentTreatment
Tricyclics: Doxepin, AmitriptylineTricyclics: Doxepin, Amitriptyline Antihistamines: 1Antihistamines: 1stst line: line: Promethazine (Phenergan)Promethazine (Phenergan) Diphenhydramine (Benadryl)Diphenhydramine (Benadryl) Hydroxyzine (Atarax, Vistaril) Hydroxyzine (Atarax, Vistaril) Azatadine (Rynatan)Azatadine (Rynatan)
TreatmentTreatment
Non sedating antihistamines:Non sedating antihistamines: Loratidine (Claritin, Alavert)Loratidine (Claritin, Alavert) Fexofenadine (Allegra)Fexofenadine (Allegra) Acrivastine (Semprex D)Acrivastine (Semprex D) Cetirizine (Zyrtec)Cetirizine (Zyrtec)
TreatmentTreatment
Bag of iceBag of ice Hot water bottleHot water bottle ““Caine” preparations good for short Caine” preparations good for short
term relief but often become term relief but often become sensitizerssensitizers
Doxepin cream or PramoxineDoxepin cream or Pramoxine Sarna (menthol lotion)Sarna (menthol lotion)
TX: Severe RecalcitrantTX: Severe Recalcitrant
HIV, CRF, Liver failureHIV, CRF, Liver failure IV Lidocaine – limited by hypotension IV Lidocaine – limited by hypotension
and short duration of actionand short duration of action Pruritis of cholestasis:Pruritis of cholestasis: NaloxoneNaloxone Ondansetron 8mg per dayOndansetron 8mg per day
Paroxysmal PruritisParoxysmal Pruritis
Sudden in onset, irresistably severe, Sudden in onset, irresistably severe, intense pleasure with scratchingintense pleasure with scratching
LSC, AD, Nummular, DH, LSC, AD, Nummular, DH, Neurodermatitis, Eosinophilic Neurodermatitis, Eosinophilic folliculitis, Uremia, Prurigo, Prurigo folliculitis, Uremia, Prurigo, Prurigo NodularisNodularis
Labwork/Internal CausesLabwork/Internal Causes
CMP: Liver disease, Renal Failure, DM CMP: Liver disease, Renal Failure, DM IIII
Hepatits Panel: Hepatitis CHepatits Panel: Hepatitis C TSH: Thyroid (high or low)TSH: Thyroid (high or low) CBC: Anemia, Polycythemia Vera, CBC: Anemia, Polycythemia Vera,
Leukemia, Myeloma, Hodgkins Leukemia, Myeloma, Hodgkins Lymphoma, Intestinal ParasitesLymphoma, Intestinal Parasites
CXR: R/O Cancer CXR: R/O Cancer
Internal Causes of PruritisInternal Causes of Pruritis
10-25% of Hodgkins patients have 10-25% of Hodgkins patients have itch (continuous and at times itch (continuous and at times burning) as a symptom, and for 7% it burning) as a symptom, and for 7% it is the FIRST presenting symptom.is the FIRST presenting symptom.
3% to 47% of patients with 3% to 47% of patients with generalized pruritis unexplained by generalized pruritis unexplained by skin lesions may have internal skin lesions may have internal cancer.cancer.
Polycythemia VeraPolycythemia Vera
1/3 of these patients report pruritis1/3 of these patients report pruritis Pruritis is induced by temperature Pruritis is induced by temperature
changeschanges Treatment: Low dose ASA, PUVA, Treatment: Low dose ASA, PUVA,
Interferon alpha-2b, chemotherapy.Interferon alpha-2b, chemotherapy. NOTE: Antihistamines ineffectiveNOTE: Antihistamines ineffective
Biliary PruritisBiliary Pruritis
Chronic liver disease with obstructive Chronic liver disease with obstructive jaundice is the causejaundice is the cause
20-50% of pts with jaundice have 20-50% of pts with jaundice have pruritispruritis
Central mechanism: elevated CNS opiod Central mechanism: elevated CNS opiod peptide levels -> Naloxone treatmentpeptide levels -> Naloxone treatment
Bile acid levels do not correlate with Bile acid levels do not correlate with severity of pruritisseverity of pruritis
Primary Biliary CirrhosisPrimary Biliary Cirrhosis Women > 30Women > 30 Starts insidiously, Starts insidiously,
becomes intolerablebecomes intolerable Jaundice with Jaundice with
striking melanotic striking melanotic hyper-pigmentation hyper-pigmentation of the entire skin - of the entire skin - except for a except for a “butterfly area” of “butterfly area” of normal pigmentation normal pigmentation in the upper back in the upper back
Primary Biliary CirrhosisPrimary Biliary Cirrhosis Xanthomas also seen.Xanthomas also seen. Antimitochondrial Antimitochondrial
antibody test +antibody test + Alk. Phos, Alk. Phos,
Ceruloplasmin, Ceruloplasmin, Bilirubin, Cholesterol Bilirubin, Cholesterol
Tx: Cholestyramine, Tx: Cholestyramine, Rifampin, Naloxone, Rifampin, Naloxone, SAM, Prednisolone, SAM, Prednisolone, Colchicine, Ursodeoxy-Colchicine, Ursodeoxy-cholic acid, Liver cholic acid, Liver TransplantTransplant
Renal Failure/Uremic PruritisRenal Failure/Uremic Pruritis MC internal cause of pruritisMC internal cause of pruritis 50-90% of dialysis patients within 6 mos.50-90% of dialysis patients within 6 mos. Dialysis related = episodicDialysis related = episodic Uremic = generalized, intractable, severeUremic = generalized, intractable, severe Causes are multifactorialCauses are multifactorial TX: Regular dialysis, Epoetin, Emollients, TX: Regular dialysis, Epoetin, Emollients,
Topical Capsaicin, Antihistamines, Topical Capsaicin, Antihistamines, Cholestyramine, UVB, ThalidomideCholestyramine, UVB, Thalidomide
XEROSIS AND PRURITIS IN PATIENT WITH CHRONIC RENAL FAILURE ON HEMODIALYSIS
Winter ItchWinter Itch AKA Asteatotic AKA Asteatotic
Eczema, Eczema Eczema, Eczema CraqueleCraquele
Cause: frequent Cause: frequent harsh bathing in harsh bathing in winterwinter
ElderlyElderly TX: Lubrication of TX: Lubrication of
skin immediately skin immediately after bathingafter bathing
Lac-Hydrin 12%Lac-Hydrin 12%
Pruritis AniPruritis Ani
Neurodermatitis, paroxysmalNeurodermatitis, paroxysmal Requires ruling out other causes:Requires ruling out other causes: Allergic contact from creams appliedAllergic contact from creams applied Irritation: spicy foods, cathartics, Irritation: spicy foods, cathartics,
leakage, may need change in dietleakage, may need change in diet Fungal cultures, KOH, DTM, Nickersons, Fungal cultures, KOH, DTM, Nickersons,
Wood’s lamp exam, Bacterial culture.Wood’s lamp exam, Bacterial culture. Stool for Ova and Parasites, Pinworm.Stool for Ova and Parasites, Pinworm. Anal gonorrhea frequently overlookedAnal gonorrhea frequently overlooked
Pruritis AniPruritis Ani
TreatmentTreatment Meticulous toilet care using soft cellulose Meticulous toilet care using soft cellulose
tissue paper and whenever possible tissue paper and whenever possible washed with mild soap and water.washed with mild soap and water.
Wet toilet tissue preferredWet toilet tissue preferred Tucks, Balneol, PramosoneTucks, Balneol, Pramosone Allow cultures to direct specific therapyAllow cultures to direct specific therapy
Pruritis Scroti Pruritis Scroti
LSC variantLSC variant Infections possible but unlikelyInfections possible but unlikely Candida produces burn more than itchCandida produces burn more than itch Low potency steroids only as skin here Low potency steroids only as skin here
can get steroid addictedcan get steroid addicted Pramosone (Pramoxine) , Zonalon Pramosone (Pramoxine) , Zonalon
(Doxepin)(Doxepin)
Pruritis VulvaePruritis Vulvae
MC cause is non-specific dermatitisMC cause is non-specific dermatitis Candida infection common during Candida infection common during
pregnancy/post oral antibiotics pregnancy/post oral antibiotics Consider LS&A, Dysesthetic Consider LS&A, Dysesthetic
Vulvodynia and Psoriasis. Also Vulvodynia and Psoriasis. Also Trichomonas Treatment same as Trichomonas Treatment same as Pruritis ScrotiPruritis Scroti
Treatment failure should prompt Treatment failure should prompt referral or biopsy.referral or biopsy.
Puncta Pruritica (Itchy Puncta Pruritica (Itchy Points)Points)
One or two intensely itching spots in One or two intensely itching spots in clinically normal skin, sometimes clinically normal skin, sometimes followed by the appearance of SK.followed by the appearance of SK.
Treatment CRYO, Curettage or Punch Treatment CRYO, Curettage or Punch biopsybiopsy
Aquagenic Pruritis & Aquagenic Pruritis & AquadyniaAquadynia
AP provoked by water at any temperature AP provoked by water at any temperature usually with family history of the sameusually with family history of the same
Degranulation of mast cells within minutesDegranulation of mast cells within minutes Aquadynia is a “burning” variant of APAquadynia is a “burning” variant of AP Assoc: polycythemia vera, Assoc: polycythemia vera,
hypereosinophilic synd, JXG, hypereosinophilic synd, JXG, myelodysplastic synd. myelodysplastic synd.
TX: OAH, Prednisone, OS, Capsaicin, NTG, TX: OAH, Prednisone, OS, Capsaicin, NTG, Propranolol, ClonidinePropranolol, Clonidine
Scalp PruritisScalp Pruritis
Elderly patientsElderly patients Non-scaling, non-erythematous, without Non-scaling, non-erythematous, without
excoriations (cannot diagnose SD, PV or excoriations (cannot diagnose SD, PV or LSC)LSC)
Probably a chronic folliculitis of some Probably a chronic folliculitis of some sortsort
Cause unknown in most casesCause unknown in most cases TX: difficult, tar, SA, TS, IL steroids, OAHTX: difficult, tar, SA, TS, IL steroids, OAH
Drug Induced PruritisDrug Induced Pruritis
ChloroquineChloroquine AmiodaroneAmiodarone Hydroxyethyl Starch or HES (Volume Hydroxyethyl Starch or HES (Volume
expander, human plasma substitute)expander, human plasma substitute)
Prurigo/Prurigo NodularisPrurigo/Prurigo Nodularis
Extremities – Papules/nodules, firm, Extremities – Papules/nodules, firm, verrucous in late lesionsverrucous in late lesions
Bleeding, scarring, chronic, Bleeding, scarring, chronic, paroxysmal.paroxysmal.
Severe but itch is restricted to lesions Severe but itch is restricted to lesions themselvesthemselves
Bx R/O PLEVA, DH, TAD, Scabies, AD, Bx R/O PLEVA, DH, TAD, Scabies, AD, Insect bite, Papular urticaria, Contact.Insect bite, Papular urticaria, Contact.
Treatment and Etiology: Unknown.Treatment and Etiology: Unknown.
Prurigo Nodularis with secondary bacterial infection
Prurigo PigmentosaPrurigo Pigmentosa Rare, etiology unknownRare, etiology unknown Japanese women in spring and summerJapanese women in spring and summer Sudden onset of erythematous papules Sudden onset of erythematous papules
that leave reticulated that leave reticulated hyperpigmentation when they heal, hyperpigmentation when they heal, often recurrent.often recurrent.
upper back, nape, clavicular & chestupper back, nape, clavicular & chest H&E lichenoid infiltrate w/ psoriasiform H&E lichenoid infiltrate w/ psoriasiform
hyperplasiahyperplasia Minocycline 100mg BID, Dapsone.Minocycline 100mg BID, Dapsone.
Papuloerythroderma of OfujiPapuloerythroderma of Ofuji Rare, JapanRare, Japan Widespread flat topped papules that Widespread flat topped papules that
strikingly spare the skin folds, strikingly spare the skin folds, producing bands of uninvolved cutis, producing bands of uninvolved cutis, known as the DECK CHAIR SIGNknown as the DECK CHAIR SIGN
PATH: dense lymphohist. infilt. w/ PATH: dense lymphohist. infilt. w/ eosinophils in papillary dermiseosinophils in papillary dermis
Assoc: HIV, lymphomaAssoc: HIV, lymphoma TX: Oral steroidsTX: Oral steroids
Lichen Simplex ChronicusLichen Simplex Chronicus
AKA Neurodermatitis CircumsciptaAKA Neurodermatitis Circumscipta Result of long term rubbing/scratchingResult of long term rubbing/scratching Striae form a criss-cross pattern, and Striae form a criss-cross pattern, and
between them is a mosaic of flat topped, between them is a mosaic of flat topped, shiny smooth quadrilateral facetsshiny smooth quadrilateral facets
Paroxysmal, neck, wrists, anklesParoxysmal, neck, wrists, ankles TX: TS, IL, Occlusion, Zonalon, Capsaicin TX: TS, IL, Occlusion, Zonalon, Capsaicin
PSYCHODERMATOLOGYPSYCHODERMATOLOGY
Onychophagia – nail bitingOnychophagia – nail biting Dermatophagia – biting one’s own skinDermatophagia – biting one’s own skin Lip licking “clown mouth make-up”Lip licking “clown mouth make-up” OCD – complusive hand washingOCD – complusive hand washing Bulimia – crusted papules on dorsum of Bulimia – crusted papules on dorsum of
hands from cuts by teethhands from cuts by teeth Fist clenching – fingertip swelling and Fist clenching – fingertip swelling and
ecchymosis, subungual hemorrhageecchymosis, subungual hemorrhage
Irritant Hand Dermatitis Irritant Hand Dermatitis PearlPearl
OCD is often the cause, repetitive OCD is often the cause, repetitive hand- washinghand- washing
Growing body of evidence supporting Growing body of evidence supporting a neurobiological cause of diseasea neurobiological cause of disease
Treatment with Clomipramine, Treatment with Clomipramine, Fluoxetine, Fluvoxamine, Sertraline, Fluoxetine, Fluvoxamine, Sertraline, Paroxetine, VenlafaxineParoxetine, Venlafaxine
Behavioral therapyBehavioral therapy
Delusions of ParasitosisDelusions of Parasitosis ““Matchbox sign”Matchbox sign” Belief is “fixed” in patient’s mind.Belief is “fixed” in patient’s mind. Middle aged or elderly women MC.Middle aged or elderly women MC. Work-up: Bx, CBC, UA, LFTs, TSH, Work-up: Bx, CBC, UA, LFTs, TSH,
Iron, B-12, Folate, Electolytes.Iron, B-12, Folate, Electolytes. Psych consult usually refused.Psych consult usually refused. Pimozide 1 to 12mg plus Cogentin or Pimozide 1 to 12mg plus Cogentin or
Benadryl to allay Extrapyramidal SEs. Benadryl to allay Extrapyramidal SEs.
Neurotic ExcoriationsNeurotic Excoriations
Patient often unaware they are doing itPatient often unaware they are doing it Compulsion: Tension -> Picking -> ReliefCompulsion: Tension -> Picking -> Relief Acne excorie de jeune fillesAcne excorie de jeune filles MC assocs: depression, OCD and anxietyMC assocs: depression, OCD and anxiety TOC: Doxepin, Buspar.TOC: Doxepin, Buspar. Co-manage with a therapist to manage Co-manage with a therapist to manage
self-aggressive behaviors.self-aggressive behaviors.
ACNE EXCORIE
Factitious DermatitisFactitious Dermatitis Self Inflicted for secondary gain Self Inflicted for secondary gain
sympathy, escape responsibility, sympathy, escape responsibility, disability.disability.
Distinctive clear cut bizarre appearance.Distinctive clear cut bizarre appearance. Usually linear & arranged symmetricallyUsually linear & arranged symmetrically Chemical burns, injected foreign Chemical burns, injected foreign
material, sharp instruments, tight cords material, sharp instruments, tight cords lymphedema, injection of air lymphedema, injection of air crepitus/subutaneous emphysema, non-crepitus/subutaneous emphysema, non-healing post-op wound.healing post-op wound.
Factitious DermatitisFactitious Dermatitis
How do you prove the diagnosis ?How do you prove the diagnosis ? Occlusive dressing, ie cast, unna Occlusive dressing, ie cast, unna
boot.boot. Biopsy with polarization to r/o FB.Biopsy with polarization to r/o FB. If the patient is hospitalized, a If the patient is hospitalized, a
resourceful nurse may be helpful.resourceful nurse may be helpful. Psych consult usually refused.Psych consult usually refused. DOC: Pimozide, Prozac.DOC: Pimozide, Prozac.
TrichotillomaniaTrichotillomania Varying lengths of broken hairs present Varying lengths of broken hairs present
and lack of nail pitting helps R/O AA.and lack of nail pitting helps R/O AA. MC: frontal scalp, eyebrows, eyelashes.MC: frontal scalp, eyebrows, eyelashes. Onychophagy / nail biting may be Onychophagy / nail biting may be
present.present. Etio: Psychosocial stress in the familyEtio: Psychosocial stress in the family Bx: perifollicular hemorrhage, pigment Bx: perifollicular hemorrhage, pigment
casts, trichomalaciacasts, trichomalacia Assoc: Depression, OCD, AnxietyAssoc: Depression, OCD, Anxiety Child Psychiatrist consult, Anafranil, Child Psychiatrist consult, Anafranil,
Prozac.Prozac.
Twisted distorted hair shaft
“TRICHOMALACIA”
Pigmented follicular cast
Cutaneous neurosis characterized by Cutaneous neurosis characterized by an uncontrollable desire to rub or an uncontrollable desire to rub or pinch oneself to form bruised areas pinch oneself to form bruised areas on the skin, sometimes as a defense on the skin, sometimes as a defense against pain elsewhere.against pain elsewhere.
DermatothlasiaDermatothlasia
BromidosiphobiaBromidosiphobia Neurosis/conviction that one’s sweat Neurosis/conviction that one’s sweat
is malodorous/repugnant/keeps is malodorous/repugnant/keeps people away.people away.
Patient unable to accept evidence to Patient unable to accept evidence to the contrarythe contrary
Early symptom of schizophreniaEarly symptom of schizophrenia Males, average age is 25.Males, average age is 25. PimozidePimozide
Body Dysmorphic DisorderBody Dysmorphic Disorder
Delusion of having an ugly body partDelusion of having an ugly body part Obsessional features and depressionObsessional features and depression Risk of suicideRisk of suicide 1% of US population1% of US population SSRIs helpfulSSRIs helpful
Glossodynia/Burning TongueGlossodynia/Burning Tongue
Post-menopausal womenPost-menopausal women Constant burning of tongue, mouth, Constant burning of tongue, mouth,
lipslips No objective findingsNo objective findings No known etiology – Sjogren’s?, B-No known etiology – Sjogren’s?, B-
12? Folate?, Iron? DM II?12? Folate?, Iron? DM II? TOC: Mood altering drugs.TOC: Mood altering drugs.
Scalp DysesthesiaScalp Dysesthesia
Pain, burning or pruritic symptomsPain, burning or pruritic symptoms No objective findingsNo objective findings Psychiatric overlayPsychiatric overlay TOC: AntidepressantsTOC: Antidepressants
Vulvodynia (Burning Vulva Vulvodynia (Burning Vulva Synd)Synd)
1) Severe pain on vestibular touch or 1) Severe pain on vestibular touch or attempted vaginal entryattempted vaginal entry
2) Tenderness to pressure localized to 2) Tenderness to pressure localized to vulvar vestibulevulvar vestibule
3) Vulvar erythema of varying degrees3) Vulvar erythema of varying degrees Patients are white, nulliparous, age 32.Patients are white, nulliparous, age 32. R/O candida, HPV, Trichomonas, R/O candida, HPV, Trichomonas,
contactcontact
Vulvodynia (Burning Vulva Vulvodynia (Burning Vulva Synd)Synd)
Treatment: Lubricant prior to Treatment: Lubricant prior to intercourseintercourse
Topical anestheticsTopical anesthetics Low oxalate dietLow oxalate diet Calcium 200mg and Citrate 950mg Calcium 200mg and Citrate 950mg
dailydaily Alpha INF injectionsAlpha INF injections Surgical excision Surgical excision
Notalgia ParestheticaNotalgia Paresthetica
Unilateral infrascapularUnilateral infrascapular Pruritis, burning pain, hyperalgesia Pruritis, burning pain, hyperalgesia
tendernesstenderness Pigmented patch localized to 2Pigmented patch localized to 2ndnd to 6 to 6thth
thoracic spinal nerves thoracic spinal nerves Macular amyloidosis may be found on Bx.Macular amyloidosis may be found on Bx. Topical Capsaicin, Anesthetics & SteroidsTopical Capsaicin, Anesthetics & Steroids Paravertebral blocksParavertebral blocks
Meralgia Paresthetica Meralgia Paresthetica
Aka Roth-Bernhardt DiseaseAka Roth-Bernhardt Disease Persistent numbness with periodic Persistent numbness with periodic
lancinating pain of anterolateral thigh.lancinating pain of anterolateral thigh. Lateral femoral cutaneous nerve.Lateral femoral cutaneous nerve. Middle aged obese menMiddle aged obese men Surgical decompression of lateral Surgical decompression of lateral
femoral cutaneous nerve.femoral cutaneous nerve.
Complex Regional Pain Complex Regional Pain Synd. Synd.
Aka Reflex Sympathetic DystrophyAka Reflex Sympathetic Dystrophy Burning Pain, Hyperesthesia and Burning Pain, Hyperesthesia and
trophic disturbances due to injured trophic disturbances due to injured peripheral nerve, usually upper peripheral nerve, usually upper extremityextremity
Skin becomes shiny, cold, profusely Skin becomes shiny, cold, profusely sweaty, cracked, edematous.sweaty, cracked, edematous.
Late: atrophy, flexion contractures and Late: atrophy, flexion contractures and osteoporosis.osteoporosis.
CRPS Type II has a precipitating eventCRPS Type II has a precipitating event Tx: Neurologist and AnesthesiologistTx: Neurologist and Anesthesiologist
Reflex Sympathetic Reflex Sympathetic DystrophyDystrophy
Trigeminal Trophic LesionsTrigeminal Trophic Lesions After rhizotomy or alcohol injection to After rhizotomy or alcohol injection to
trigeminal nerve for Tic Doloureauxtrigeminal nerve for Tic Doloureaux Slowly enlarging uninflamed ulcer may Slowly enlarging uninflamed ulcer may
appear on the cheek beside the nasal appear on the cheek beside the nasal alaala
Onset: wks-yrs s/p trigeminal nerve Onset: wks-yrs s/p trigeminal nerve injury.injury.
Etio: Self-inflicted trauma to numb skinEtio: Self-inflicted trauma to numb skin Postencephalic Trophic UlcerPostencephalic Trophic Ulcer – nose s/p – nose s/p
encephalitis encephalitis
Trigeminal Trophic LesionsTrigeminal Trophic Lesions
Malum Perforans PedisMalum Perforans Pedis
Aka Chronic Neurotrophic Ulcer
Malum Perforans PedisMalum Perforans Pedis
Assoc with denervating diseases such Assoc with denervating diseases such as tabes dorsalis, leprosy, as tabes dorsalis, leprosy, arteriosclerosis, diabetes.arteriosclerosis, diabetes.
Loss of pain sensation at a site of Loss of pain sensation at a site of repeated trauma- usually ball of footrepeated trauma- usually ball of foot
Begins as circumscribed Begins as circumscribed hyperkeratosis -> sloughs, necrosis, hyperkeratosis -> sloughs, necrosis, infection, osteomyelitisinfection, osteomyelitis
Tx: offload pressure, debridementTx: offload pressure, debridement
Sciatic Nerve InjurySciatic Nerve Injury
Etio: improper injection of buttocksEtio: improper injection of buttocks Foot drop, skin becomes shiny, thin Foot drop, skin becomes shiny, thin
and often edematousand often edematous Older patients more susceptible due to Older patients more susceptible due to
decreased muscle massdecreased muscle mass Injections should be upper outer Injections should be upper outer
quadrant of buttockquadrant of buttock Surgical exploration of sciatic nerve Surgical exploration of sciatic nerve
helpfulhelpful
Riley Day SyndromeRiley Day Syndrome Aka Familial DysautonomiaAka Familial Dysautonomia Defective lacrimation, excessive Defective lacrimation, excessive
sweating, drooling and transient sweating, drooling and transient truncal erythema.truncal erythema.
Acrocyanosis of the hands.Acrocyanosis of the hands. Absence of fungiform and circumvallate Absence of fungiform and circumvallate
papillae of the tongue.papillae of the tongue. Scalp feels ticklish when stroked lightlyScalp feels ticklish when stroked lightly Decreased pain sensationDecreased pain sensation Impaired temperature and blood Impaired temperature and blood
pressure regulationpressure regulation
Riley Day SyndromeRiley Day Syndrome Schirmer test + for lacrimal dysfunctionSchirmer test + for lacrimal dysfunction Intradermal Histamine – diminished flare.Intradermal Histamine – diminished flare. Hand immersion in H2O -> red mottling.Hand immersion in H2O -> red mottling. Increased serum ratio of DOPA to Increased serum ratio of DOPA to
dihydroxyphenylglycol.dihydroxyphenylglycol. Etio:“complex catecholamine Etio:“complex catecholamine
interactions”interactions” Decreased unmyelinated/small Decreased unmyelinated/small
myelinated neuronsmyelinated neurons Treatment is supportiveTreatment is supportive
SyringomeliaSyringomelia
Aka Morvan’s DiseaseAka Morvan’s Disease Etio: expansion of spinal cord canal Etio: expansion of spinal cord canal
compressing spinal nerves.compressing spinal nerves. Upper extremities and fingersUpper extremities and fingers Insidious onset of weakness, Insidious onset of weakness,
hyperhidrosis, sensory disturbances hyperhidrosis, sensory disturbances especially thumb and forefinger.especially thumb and forefinger.
Pain and temperature lost, but tactile Pain and temperature lost, but tactile ok.ok.
SyringomeliaSyringomelia
Asymmetric scalp hair growth with a Asymmetric scalp hair growth with a sharp midline demarcationsharp midline demarcation
Hypertrophy of limbsHypertrophy of limbs Derm Ddx: LeprosyDerm Ddx: Leprosy Unlike leprosy, syringomelia does not Unlike leprosy, syringomelia does not
interfere with sweating or block the interfere with sweating or block the flare around a histamine whealflare around a histamine wheal
Congenital Sensory Congenital Sensory NeuropathyNeuropathy
Insensitivity to pain
Anhidrosis
Sense of touch intact
Recurrent acral ulcers
Repeated injuries to hands result in mutilation
Treatment: avoid trauma to hands
the endthe end