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Pruritis and Neurocutaneous Dermatoses. KCOM/Texas Dermatology Residency Consortium July 2003. Pruritis. Fine unmyelinated C fibers Also control touch, temperature and pain Subepidermal to lateral spinothalamic tract Spinothalamic tract to thalamus Thalamus to sensory cortex.

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pruritis and neurocutaneous dermatoses

Pruritis and Neurocutaneous Dermatoses

KCOM/Texas Dermatology Residency Consortium

July 2003

  • Fine unmyelinated C fibers
  • Also control touch, temperature and pain
  • Subepidermal to lateral spinothalamic tract
  • Spinothalamic tract to thalamus
  • Thalamus to sensory cortex
central itch perception
Central Itch Perception
  • Sedation effect may decrease “central” itch perception regarding antihistamines like Atarax.
  • Histamine is a “peripheral” mediator of itch perception
  • Naloxone is a “central” opiod antagonist for patients with pruritic cholestasis.
mediators of pruritis
Mediators of Pruritis
  • Histamine, Kinins, Proteases
  • Prostaglandin E lowers threshold for histamine induced pruritis
  • Enkephalins, pentapeptides which bind to opiate receptors in the brain modulate pain and itching centrally.
  • Interleukins implicated in AD.
substance p
Substance P
  • 11 amino acid peptide implicated as causing itching in some disorders.
  • Depletes cutaneous nociceptor nerve endings of Substance P after repeated topical application.
5 hydroxytryptamine 5 ht
5-hydroxytryptamine (5-HT)
  • Regulates 5-HT receptors, may be therapeutic
  • Ondansetron (Zofran) anti-emetic blocks 5-HT.
  • Therapeutic in cholestatic pruritis
variations in intensity of itch
Variations in Intensity of itch…
  • Psychological trauma
  • Stress
  • Absence of distractions
  • Anxiety, fear
  • Anatomic regions very susceptible to pruritis: ear canals, eyelids, nostrils, perianal, genital areas.
  • Tricyclics: Doxepin, Amitriptyline
  • Antihistamines: 1st line:
  • Promethazine (Phenergan)
  • Diphenhydramine (Benadryl)
  • Hydroxyzine (Atarax, Vistaril)
  • Azatadine (Rynatan)
  • Non sedating antihistamines:
  • Loratidine (Claritin, Alavert)
  • Fexofenadine (Allegra)
  • Acrivastine (Semprex D)
  • Cetirizine (Zyrtec)
  • Bag of ice
  • Hot water bottle
  • “Caine” preparations good for short term relief but often become sensitizers
  • Doxepin cream or Pramoxine
  • Sarna (menthol lotion)
tx severe recalcitrant
TX: Severe Recalcitrant
  • HIV, CRF, Liver failure
  • IV Lidocaine – limited by hypotension and short duration of action
  • Pruritis of cholestasis:
  • Naloxone
  • Ondansetron 8mg per day
paroxysmal pruritis
Paroxysmal Pruritis
  • Sudden in onset, irresistably severe, intense pleasure with scratching
  • LSC, AD, Nummular, DH, Neurodermatitis, Eosinophilic folliculitis, Uremia, Prurigo, Prurigo Nodularis
labwork internal causes
Labwork/Internal Causes
  • CMP: Liver disease, Renal Failure, DM II
  • Hepatits Panel: Hepatitis C
  • TSH: Thyroid (high or low)
  • CBC: Anemia, Polycythemia Vera, Leukemia, Myeloma, Hodgkins Lymphoma, Intestinal Parasites
  • CXR: R/O Cancer
internal causes of pruritis
Internal Causes of Pruritis
  • 10-25% of Hodgkins patients have itch (continuous and at times burning) as a symptom, and for 7% it is the FIRST presenting symptom.
  • 3% to 47% of patients with generalized pruritis unexplained by skin lesions may have internal cancer.
polycythemia vera
Polycythemia Vera
  • 1/3 of these patients report pruritis
  • Pruritis is induced by temperature changes
  • Treatment: Low dose ASA, PUVA, Interferon alpha-2b, chemotherapy.
  • NOTE: Antihistamines ineffective
biliary pruritis
Biliary Pruritis
  • Chronic liver disease with obstructive jaundice is the cause
  • 20-50% of pts with jaundice have pruritis
  • Central mechanism: elevated CNS opiod peptide levels -> Naloxone treatment
  • Bile acid levels do not correlate with severity of pruritis
primary biliary cirrhosis
Primary Biliary Cirrhosis
  • Women > 30
  • Starts insidiously, becomes intolerable
  • Jaundice with striking melanotic hyper-pigmentation of the entire skin - except for a “butterfly area” of normal pigmentation in the upper back 
primary biliary cirrhosis18
Primary Biliary Cirrhosis
  • Xanthomas also seen.
  • Antimitochondrial antibody test +
  • Alk. Phos, Ceruloplasmin, Bilirubin, Cholesterol
  • Tx: Cholestyramine, Rifampin, Naloxone, SAM, Prednisolone, Colchicine, Ursodeoxy-cholic acid, Liver Transplant
renal failure uremic pruritis
Renal Failure/Uremic Pruritis
  • MC internal cause of pruritis
  • 50-90% of dialysis patients within 6 mos.
  • Dialysis related = episodic
  • Uremic = generalized, intractable, severe
  • Causes are multifactorial
  • TX: Regular dialysis, Epoetin, Emollients, Topical Capsaicin, Antihistamines, Cholestyramine, UVB, Thalidomide
winter itch
Winter Itch
  • AKA Asteatotic Eczema, Eczema Craquele
  • Cause: frequent harsh bathing in winter
  • Elderly
  • TX: Lubrication of skin immediately after bathing
  • Lac-Hydrin 12%
pruritis ani
Pruritis Ani
  • Neurodermatitis, paroxysmal
  • Requires ruling out other causes:
  • Allergic contact from creams applied
  • Irritation: spicy foods, cathartics, leakage, may need change in diet
  • Fungal cultures, KOH, DTM, Nickersons, Wood’s lamp exam, Bacterial culture.
  • Stool for Ova and Parasites, Pinworm.
  • Anal gonorrhea frequently overlooked
pruritis ani23
Pruritis Ani
  • Treatment
  • Meticulous toilet care using soft cellulose tissue paper and whenever possible washed with mild soap and water.
  • Wet toilet tissue preferred
  • Tucks, Balneol, Pramosone
  • Allow cultures to direct specific therapy
pruritis scroti
Pruritis Scroti
  • LSC variant
  • Infections possible but unlikely
  • Candida produces burn more than itch
  • Low potency steroids only as skin here can get steroid addicted
  • Pramosone (Pramoxine) , Zonalon (Doxepin)
pruritis vulvae
Pruritis Vulvae
  • MC cause is non-specific dermatitis
  • Candida infection common during pregnancy/post oral antibiotics
  • Consider LS&A, Dysesthetic Vulvodynia and Psoriasis. Also Trichomonas Treatment same as Pruritis Scroti
  • Treatment failure should prompt referral or biopsy.
puncta pruritica itchy points
Puncta Pruritica (Itchy Points)
  • One or two intensely itching spots in clinically normal skin, sometimes followed by the appearance of SK.
  • Treatment CRYO, Curettage or Punch biopsy
aquagenic pruritis aquadynia
Aquagenic Pruritis & Aquadynia
  • AP provoked by water at any temperature usually with family history of the same
  • Degranulation of mast cells within minutes
  • Aquadynia is a “burning” variant of AP
  • Assoc: polycythemia vera, hypereosinophilic synd, JXG, myelodysplastic synd.
  • TX: OAH, Prednisone, OS, Capsaicin, NTG, Propranolol, Clonidine
scalp pruritis
Scalp Pruritis
  • Elderly patients
  • Non-scaling, non-erythematous, without excoriations (cannot diagnose SD, PV or LSC)
  • Probably a chronic folliculitis of some sort
  • Cause unknown in most cases
  • TX: difficult, tar, SA, TS, IL steroids, OAH
drug induced pruritis
Drug Induced Pruritis
  • Chloroquine
  • Amiodarone
  • Hydroxyethyl Starch or HES (Volume expander, human plasma substitute)
prurigo prurigo nodularis
Prurigo/Prurigo Nodularis
  • Extremities – Papules/nodules, firm, verrucous in late lesions
  • Bleeding, scarring, chronic, paroxysmal.
  • Severe but itch is restricted to lesions themselves
  • Bx R/O PLEVA, DH, TAD, Scabies, AD, Insect bite, Papular urticaria, Contact.
  • Treatment and Etiology: Unknown.
prurigo pigmentosa
Prurigo Pigmentosa
  • Rare, etiology unknown
  • Japanese women in spring and summer
  • Sudden onset of erythematous papules that leave reticulated hyperpigmentation when they heal, often recurrent.
  • upper back, nape, clavicular & chest
  • H&E lichenoid infiltrate w/ psoriasiform hyperplasia
  • Minocycline 100mg BID, Dapsone.
papuloerythroderma of ofuji
Papuloerythroderma of Ofuji
  • Rare, Japan
  • Widespread flat topped papules that strikingly spare the skin folds, producing bands of uninvolved cutis, known as the DECK CHAIR SIGN
  • PATH: dense lymphohist. infilt. w/ eosinophils in papillary dermis
  • Assoc: HIV, lymphoma
  • TX: Oral steroids
lichen simplex chronicus
Lichen Simplex Chronicus
  • AKA Neurodermatitis Circumscipta
  • Result of long term rubbing/scratching
  • Striae form a criss-cross pattern, and between them is a mosaic of flat topped, shiny smooth quadrilateral facets
  • Paroxysmal, neck, wrists, ankles
  • TX: TS, IL, Occlusion, Zonalon, Capsaicin
  • Onychophagia – nail biting
  • Dermatophagia – biting one’s own skin
  • Lip licking “clown mouth make-up”
  • OCD – complusive hand washing
  • Bulimia – crusted papules on dorsum of hands from cuts by teeth
  • Fist clenching – fingertip swelling and ecchymosis, subungual hemorrhage
irritant hand dermatitis pearl
Irritant Hand Dermatitis Pearl
  • OCD is often the cause, repetitive hand- washing
  • Growing body of evidence supporting a neurobiological cause of disease
  • Treatment with Clomipramine, Fluoxetine, Fluvoxamine, Sertraline, Paroxetine, Venlafaxine
  • Behavioral therapy
delusions of parasitosis
Delusions of Parasitosis
  • “Matchbox sign”
  • Belief is “fixed” in patient’s mind.
  • Middle aged or elderly women MC.
  • Work-up: Bx, CBC, UA, LFTs, TSH, Iron, B-12, Folate, Electolytes.
  • Psych consult usually refused.
  • Pimozide 1 to 12mg plus Cogentin or Benadryl to allay Extrapyramidal SEs.
neurotic excoriations
Neurotic Excoriations
  • Patient often unaware they are doing it
  • Compulsion: Tension -> Picking -> Relief
  • Acne excorie de jeune filles
  • MC assocs: depression, OCD and anxiety
  • TOC: Doxepin, Buspar.
  • Co-manage with a therapist to manage self-aggressive behaviors.
factitious dermatitis
Factitious Dermatitis
  • Self Inflicted for secondary gain  sympathy, escape responsibility, disability.
  • Distinctive clear cut bizarre appearance.
  • Usually linear & arranged symmetrically
  • Chemical burns, injected foreign material, sharp instruments, tight cords  lymphedema, injection of air  crepitus/subutaneous emphysema, non-healing post-op wound.
factitious dermatitis48
Factitious Dermatitis
  • How do you prove the diagnosis ?
  • Occlusive dressing, ie cast, unna boot.
  • Biopsy with polarization to r/o FB.
  • If the patient is hospitalized, a resourceful nurse may be helpful.
  • Psych consult usually refused.
  • DOC: Pimozide, Prozac.
  • Varying lengths of broken hairs present and lack of nail pitting helps R/O AA.
  • MC: frontal scalp, eyebrows, eyelashes.
  • Onychophagy / nail biting may be present.
  • Etio: Psychosocial stress in the family
  • Bx: perifollicular hemorrhage, pigment casts, trichomalacia
  • Assoc: Depression, OCD, Anxiety
  • Child Psychiatrist consult, Anafranil, Prozac.

Twisted distorted hair shaft



Cutaneous neurosis characterized by an uncontrollable desire to rub or pinch oneself to form bruised areas on the skin, sometimes as a defense against pain elsewhere.

  • Neurosis/conviction that one’s sweat is malodorous/repugnant/keeps people away.
  • Patient unable to accept evidence to the contrary
  • Early symptom of schizophrenia
  • Males, average age is 25.
  • Pimozide
body dysmorphic disorder
Body Dysmorphic Disorder
  • Delusion of having an ugly body part
  • Obsessional features and depression
  • Risk of suicide
  • 1% of US population
  • SSRIs helpful
glossodynia burning tongue
Glossodynia/Burning Tongue
  • Post-menopausal women
  • Constant burning of tongue, mouth, lips
  • No objective findings
  • No known etiology – Sjogren’s?, B-12? Folate?, Iron? DM II?
  • TOC: Mood altering drugs.
scalp dysesthesia
Scalp Dysesthesia
  • Pain, burning or pruritic symptoms
  • No objective findings
  • Psychiatric overlay
  • TOC: Antidepressants
vulvodynia burning vulva synd
Vulvodynia (Burning Vulva Synd)
  • 1) Severe pain on vestibular touch or attempted vaginal entry
  • 2) Tenderness to pressure localized to vulvar vestibule
  • 3) Vulvar erythema of varying degrees
  • Patients are white, nulliparous, age 32.
  • R/O candida, HPV, Trichomonas, contact
vulvodynia burning vulva synd60
Vulvodynia (Burning Vulva Synd)
  • Treatment: Lubricant prior to intercourse
  • Topical anesthetics
  • Low oxalate diet
  • Calcium 200mg and Citrate 950mg daily
  • Alpha INF injections
  • Surgical excision
notalgia paresthetica
Notalgia Paresthetica
  • Unilateral infrascapular
  • Pruritis, burning pain, hyperalgesia tenderness
  • Pigmented patch localized to 2nd to 6th thoracic spinal nerves
  • Macular amyloidosis may be found on Bx.
  • Topical Capsaicin, Anesthetics & Steroids
  • Paravertebral blocks
meralgia paresthetica
Meralgia Paresthetica
  • Aka Roth-Bernhardt Disease
  • Persistent numbness with periodic lancinating pain of anterolateral thigh.
  • Lateral femoral cutaneous nerve.
  • Middle aged obese men
  • Surgical decompression of lateral femoral cutaneous nerve.
complex regional pain synd
Complex Regional Pain Synd.
  • Aka Reflex Sympathetic Dystrophy
  • Burning Pain, Hyperesthesia and trophic disturbances due to injured peripheral nerve, usually upper extremity
  • Skin becomes shiny, cold, profusely sweaty, cracked, edematous.
  • Late: atrophy, flexion contractures and osteoporosis.
  • CRPS Type II has a precipitating event
  • Tx: Neurologist and Anesthesiologist
trigeminal trophic lesions
Trigeminal Trophic Lesions
  • After rhizotomy or alcohol injection to trigeminal nerve for Tic Doloureaux
  • Slowly enlarging uninflamed ulcer may appear on the cheek beside the nasal ala
  • Onset: wks-yrs s/p trigeminal nerve injury.
  • Etio: Self-inflicted trauma to numb skin
  • Postencephalic Trophic Ulcer – nose s/p encephalitis
malum perforans pedis
Malum Perforans Pedis

Aka Chronic Neurotrophic Ulcer

malum perforans pedis68
Malum Perforans Pedis
  • Assoc with denervating diseases such as tabes dorsalis, leprosy, arteriosclerosis, diabetes.
  • Loss of pain sensation at a site of repeated trauma- usually ball of foot
  • Begins as circumscribed hyperkeratosis -> sloughs, necrosis, infection, osteomyelitis
  • Tx: offload pressure, debridement
sciatic nerve injury
Sciatic Nerve Injury
  • Etio: improper injection of buttocks
  • Foot drop, skin becomes shiny, thin and often edematous
  • Older patients more susceptible due to decreased muscle mass
  • Injections should be upper outer quadrant of buttock
  • Surgical exploration of sciatic nerve helpful
riley day syndrome
Riley Day Syndrome
  • Aka Familial Dysautonomia
  • Defective lacrimation, excessive sweating, drooling and transient truncal erythema.
  • Acrocyanosis of the hands.
  • Absence of fungiform and circumvallate papillae of the tongue.
  • Scalp feels ticklish when stroked lightly
  • Decreased pain sensation
  • Impaired temperature and blood pressure regulation
riley day syndrome71
Riley Day Syndrome
  • Schirmer test + for lacrimal dysfunction
  • Intradermal Histamine – diminished flare.
  • Hand immersion in H2O -> red mottling.
  • Increased serum ratio of DOPA to dihydroxyphenylglycol.
  • Etio:“complex catecholamine interactions”
  • Decreased unmyelinated/small myelinated neurons
  • Treatment is supportive
  • Aka Morvan’s Disease
  • Etio: expansion of spinal cord canal compressing spinal nerves.
  • Upper extremities and fingers
  • Insidious onset of weakness, hyperhidrosis, sensory disturbances especially thumb and forefinger.
  • Pain and temperature lost, but tactile ok.
  • Asymmetric scalp hair growth with a sharp midline demarcation
  • Hypertrophy of limbs
  • Derm Ddx: Leprosy
  • Unlike leprosy, syringomelia does not interfere with sweating or block the flare around a histamine wheal
congenital sensory neuropathy
Congenital Sensory Neuropathy

Insensitivity to pain


Sense of touch intact

Recurrent acral ulcers

Repeated injuries to hands result in mutilation

Treatment: avoid trauma to hands