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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

pruritis
Pruritis
  • 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.
treatment
Treatment
  • Tricyclics: Doxepin, Amitriptyline
  • Antihistamines: 1st line:
  • Promethazine (Phenergan)
  • Diphenhydramine (Benadryl)
  • Hydroxyzine (Atarax, Vistaril)
  • Azatadine (Rynatan)
treatment9
Treatment
  • Non sedating antihistamines:
  • Loratidine (Claritin, Alavert)
  • Fexofenadine (Allegra)
  • Acrivastine (Semprex D)
  • Cetirizine (Zyrtec)
treatment10
Treatment
  • 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
psychodermatology
PSYCHODERMATOLOGY
  • 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.
trichotillomania
Trichotillomania
  • 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.
slide51

Twisted distorted hair shaft

“TRICHOMALACIA”

dermatothlasia
Dermatothlasia

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.

bromidosiphobia
Bromidosiphobia
  • 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
syringomelia
Syringomelia
  • 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.
syringomelia73
Syringomelia
  • 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

Anhidrosis

Sense of touch intact

Recurrent acral ulcers

Repeated injuries to hands result in mutilation

Treatment: avoid trauma to hands