Pruritus and Pruritus and Neurocutaneous Neurocutaneous

DermatosesDermatoses

Boris Ioffe, D.O.Boris Ioffe, D.O.

November 2005 November 2005

PruritusPruritus

ItchItch Affects skin, tracheal mucous membrane, Affects skin, tracheal mucous membrane,

and mucocutaneous junctionsand mucocutaneous junctions Often perceived as most unedurable Often perceived as most unedurable

symptomsymptom Feature of many systemic diseasesFeature of many systemic diseases Pruritoceptive or neurogenicPruritoceptive or neurogenic Maybe a sole symptom in the normal skinMaybe a sole symptom in the normal skin

PruritusPruritus

Mediated byMediated by Fine unmyelinated C fibersFine unmyelinated C fibers Also control touch, temperature and Also control touch, temperature and

painpain Subepidermal to lateral spinothalamic Subepidermal to lateral spinothalamic

tract 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 PruritusMediators of Pruritus

Histamine, Kinins, ProteasesHistamine, Kinins, Proteases Prostaglandin E lowers threshold for Prostaglandin E lowers threshold for

histamine induced pruritushistamine induced pruritus 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.

HistamineHistamine

Mediator of signs and symptoms of Mediator of signs and symptoms of inflammation including pruritusinflammation including pruritus

Contained in granules of mast cellsContained in granules of mast cells Acute and Chronic Urticaria and Acute and Chronic Urticaria and

MastocytosisMastocytosis Probably does not have a great role in Probably does not have a great role in

atopic dermatitis since non-sedating atopic dermatitis since non-sedating H1 blockers are not effectiveH1 blockers are not effective

Substance PSubstance P

11 amino acid peptide implicated as 11 amino acid peptide implicated as causing itching in some disorderscausing itching in some disorders

Causes pruritus, vasodilation and Causes pruritus, vasodilation and increased vascular permeabilityincreased vascular permeability

Releases histamine from dermal mast Releases histamine from dermal mast cellscells

Capsaicin depletes cutaneous Capsaicin depletes cutaneous nociceptor nerve endings of Substance nociceptor nerve endings of Substance P after repeated topical application.P after repeated topical application.

5-hydroxytryptamine (5-HT)5-hydroxytryptamine (5-HT)

Weak pruritogen, can cause pruritus Weak pruritogen, can cause pruritus via central mechanismvia central mechanism

Regulates 5-HT receptorsRegulates 5-HT receptors Ondansetron (Zofran) anti-emetic Ondansetron (Zofran) anti-emetic

blocks 5-HT.blocks 5-HT. Therapeutic in cholestatic pruritusTherapeutic in cholestatic pruritus

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) Desloratidine(Clarinex)Desloratidine(Clarinex) Cetirizine (Zyrtec)Cetirizine (Zyrtec)

Antihistamine ClassificationAntihistamine Classification

TreatmentTreatment

Topical choicesTopical choices Doxepin 5% cream – percutanoues Doxepin 5% cream – percutanoues

absorptionabsorption Capscaicin 0.025-03%Capscaicin 0.025-03% Menthol 1% lotionMenthol 1% lotion Crotamilton 10%Crotamilton 10%

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

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 PruritusParoxysmal Pruritus

Sudden in onset, irresistibly severe, Sudden in onset, irresistibly 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 pruritus1/3 of these patients report pruritus Pruritus is induced by temperature Pruritus 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 PruritusBiliary Pruritus

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 prurituspruritus

Central mechanism: elevated CNS opioid Central mechanism: elevated CNS opioid peptide levels -> Naloxone treatmentpeptide levels -> Naloxone treatment

Bile acid levels do not correlate with Bile acid levels do not correlate with severity of pruritusseverity of pruritus

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 Renal Failure/Uremic PruritusPruritus

Uremic pruritus has implication that Uremic pruritus has implication that symptoms are due to raised BUN, not truesymptoms are due to raised BUN, not true

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)

Pruritus VulvaePruritus 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 PruritusDrug Induced Pruritus

ChloroquineChloroquine AmiodaroneAmiodarone Hydroxyethyl Starch or HES (Volume Hydroxyethyl Starch or HES (Volume

expander, human plasma substitute)expander, human plasma substitute)

Prurigo NodularisPrurigo Nodularis

Chronic skin conditionChronic skin condition Multiple nodules and papules with central-Multiple nodules and papules with central-

crustcrust Often but not always due to intense pruritusOften but not always due to intense pruritus Usually affects middle-aged womenUsually affects middle-aged women Multiple excoriations and postinflammatory Multiple excoriations and postinflammatory

changes changes Face and soles are rarely effectedFace and soles are rarely effected Multiple etiologies for pruritisMultiple etiologies for pruritis

Prurigo Nodularis with secondary bacterial infection

Prurigo NodularisPrurigo Nodularis

Path: focal hyperkeratosis with non-Path: focal hyperkeratosis with non-specific dermal infiltratespecific dermal infiltrate

TreatmentTreatment Often refractoryOften refractory Stop itch-sratch cycleStop itch-sratch cycle DoxepinDoxepin Topical antipruritics(pramoxine,steroids)Topical antipruritics(pramoxine,steroids) IL steroids, cryotherapy, UVB, IL steroids, cryotherapy, UVB,

thalidomidethalidomide

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 – compulsive hand washingOCD – compulsive 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 mindBelief is “fixed” in patient’s mind Monosymptomatic Hypochandrial PsychosisMonosymptomatic Hypochandrial Psychosis Middle aged or elderly women MC.Middle aged or elderly women MC. Work-up: Bx, CBC, UA, LFTs, TSH, Iron, B-12, Work-up: Bx, CBC, UA, LFTs, TSH, Iron, B-12,

Folate, Electrolytes.Folate, Electrolytes. 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 SEBenadryl to allay Extrapyramidal SE Newer antipsychoticsNewer antipsychotics

Neurotic ExcoriationsNeurotic Excoriations

Conscious, repetitive, and uncontrollable Conscious, repetitive, and uncontrollable desire to pickdesire to pick

Patient often unaware they are doing itPatient often unaware they are doing it Compulsion: Tension -> Picking -> ReliefCompulsion: Tension -> Picking -> Relief Acne excoriee de jeune fillesAcne excoriee de jeune filles MC assocs: depression, OCD and anxietyMC assocs: depression, OCD and anxiety TOC: Doxepin, Buspar, SSRIs.TOC: Doxepin, Buspar, SSRIs. 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 /subcutaneous emphysema, crepitus /subcutaneous emphysema, non-healing post-op wound.non-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

Distressing or impairing preoccupation with Distressing or impairing preoccupation with non-existant or slight defect in appearancenon-existant or slight defect in appearance

Commonly see plastic surgeons and Commonly see plastic surgeons and dermatologistsdermatologists

Obsessional features and depressionObsessional features and depression Risk of suicideRisk of suicide 1% of US population, 10-14% patient 1% of US population, 10-14% patient

visiting dermatologists tested positivevisiting dermatologists tested positive SSRIs for OCD subtype, antipsychotics for SSRIs for OCD subtype, antipsychotics for

delusionaldelusional

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

Notalgia ParestheticaNotalgia Paresthetica

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 Trigeminal Trophic SyndromeSyndrome

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 Trigeminal Trophic SyndromeSyndrome

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