Pruritus and Neurocutaneous

Dermatoses
Chapter 4
Andrews Diseases of the Skin 12th Edition
Pruritus
• Sensation that produces the desire to scratch
• Pruritogenic stimuli – first responded by keratinocytes
• release a variety of mediators, and fine intraepidermal C-neuron filaments
• transmitted via the lateral spinothalamic tract to the brain, generate both stimulatory and inhibitory responses = sum =
quality and intensity of itch
• Elicited by normally occurring stimuli
• light touch, temperature change, and emotional stress.
• Chemical, mechanical, and electrical stimuli
• Important mediators: histamine, H4 receptor. Tryptase, eukotriene B4, prostaglandins such as PGE, acetyl-
choline, cytokines such as interleukin-31 (IL-31)
• Aggravate itching
• Heat, stress, absence of distractions, anxiety, fear
• 4 primary categories
• Pruritoceptive itch, initiated by skin disorders
• Neurogenic itch, generated in the central nervous system and caused by systemic disorders
• Neuropathic itch, caused by anatomic lesions of the central or peripheral nervous system
• Psychogenic itch, the type observed in parasitophobia
Treatment for itching
• General guidelines
• Keep cool
• Avoid hot baths or showers, wool clothing (irritant)
• Topical remedies: topical anesthetic preparations
• Benzocaine (contact sensitization)
• Pramoxine
• Lidocaine 5% ointment
• Topical lotions (menthol and camphor)
• Capsaicin – depleting substance P
• Topical steroids – only for inflammation, not for neurogenic, psychogenic, systemic-dse-related
• Calcineurin inhibitors – only for inflammation, not for neurogenic, psychogenic, systemic-dse-related
• Phototherapy with UVB, UVA, psoralen + UVA (PUVA)
• Antihistamines – 1st gen H1 (hydroxyzine, diphenhydramine) EXCEPT DOXEPIN (effective)
• Anticonvulsants – gabapentin, pregabalin
• Antidepressants – mirtazapine, SSRIs
• Thalidomide
Internal causes of pruritus: CKD
• most common systemic cause of pruritus (20-80% of px with CKD)
• generalized, intractable, and severe pruritus
• dialysis-associated pruritus: episodic, mild, or localized to the dialysis catheter site, face, or legs
• Mechanism: multifactorial
• Xerosis, secondary hyperparathyroidism, increased serum histamine levels, hypervitaminosis A, iron deficiency anemia, and neuropathy have
been implicated
• Complications
• acquired perforating disease, lichen simplex chroni- cus, and prurigo nodularis
• Tx
• Emollients
• Soaking, smearing
• γ-linolenic acid cream BID
• Gabapentin TID
• Broadband UVB phototherapy
• Nalfurafine 5ug
• Thalidomide
• Intranasal butorphanol
• IV lidocaine
• Rental transplantation
Internal causes of pruritus: Biliary pruritus
• Chronic liver disease with obstructive jaundice may cause severe generalized pruritus
• 20-50% of patients with jaundice have pruritus
• Other liver diseases
• Intrahepatic cholestasis of pregnancy
• primary sclerosing cholangitis
• hereditary cholestatic diseases such as Alagille syndrome
• Primary biliary cirrhosis
• Hepatitis C
• Cause: central mechanism
• Lysophosphatidic acid
• Tx
• Cholestyramine 4-6g daily
• Rifampin 150-300 mg.day (may cause hepatitis, use with caution)
• Naltrexone up to 50mg/day
• Sertraline 75-100 mg/day
• UVB phototherapy
• UDCA – intrahepatic cholestasis of pregnancy
• Liver transplant – definitive tx
Internal causes of pruritus: Primary biliary
cirrhosis
• occurs almost exclusively in women older than 30
• Itching may begin insidiously
• With time, extreme pruritus develops in almost 80% of patients.
• accompanied by jaundice and a striking melanotic hyperpigmentation
of the entire skin
Internal causes of pruritus: Polycythemia vera
• More than one third of patients with polycythemia vera report
pruritus
• induced by temperature changes
• Tx
• Aspirin – immediate relief from itching
• Phototherapy – PUVA, NB UVB
• Paroxetine 20mg/day
• IFN-alpha - effective for treating the underlying disease and associ- ated
pruritus
Pruritic Dermatoses: Winter Itch
• Asteatotic eczema, eczema craquelé, and xerotic eczema
• pruritus that usually first manifests and is most severe on the legs and arms.
• extension to the body is common; however, the face, scalp, groin, axillae, palms, and
soles are spared.
• skin is dry with fine flakes
• Frequent cause: frequent and lengthy bathing with plenty of soap during the winter
• Elderly, with decreased rate of epidermal water barrier repair, less productive sebaceous glands
• Low humidity in overheated rooms
• Tx
• Educating px on using soap only in axillae and inguinal area
• Lubricating skin with emollients immediately after showering
• Preparations containing lactic acid or urea applied after bathing
• “soaking and smearing” - triamcinolone, 0.025–0.1% ointment, is applied to the wet skin.
Pruritic Dermatoses: Pruritus ani
• anal or genital area
• Anal neurodermatitis
• paroxysms of violent itching, when the patient may tear at the affected area until bleeding is induced
• Allergic contact dermatitis is a common dermatologic cause or secondary complication of pruritus ani.
• medicaments, fragrance in toilet tissue, or preserva- tives in moist toilet tissue
• Irritant contact dermatitis
• from gastrointestinal contents, such as hot spices or cathartics, or failure to cleanse the area adequately after bowel movements may be causal.
• Mycotic pruritus ani is characterized by fissures and a white, sodden epidermis
• potassium hydroxide mounts: Candida albicans, Epidermophyton floccosum, or Trichophyton rubrum
• coral red fluorescence under the Wood’s light.
• Pinworm infestations
• Nocturnal pruritus
• Other intestinal parasites: Taenia solium, T. saginata, amebiasis, and Strongyloides stercoralis
• Tx
• Meticulous toilet care
• An emollient lotion (Balneol)
• Topical corticoste-roids – for noninfectious type
• Pramoxine + hydrocortisone
• Sitz baths at night + Plain petrolatum over wet skin
Pruritic Dermatoses: Pruritus scroti
• Adult scrotum is immune to dermatophyte infection, but it is a susceptible site for
circumscribed neurodermatitis (lichen simplex chronicus)
• Psychogenic pruritus - most frequent type
• Result to lichenification, can be extreme, may persist for many years despite tx
• May be complicated with infectious conditions
• Candidal infections (other fungal infections spare scrotum)
• Allergic contact dermatitis
• From topical meds – steroidal agents
• Tx
• Topical corticosteroids – WOF “addicted scrotum syndrome” from high potency topical
steroids (severe burning and redness after weaning patients off)
• Gradual tapering to less potent corticosteroids
• Topical pramoxine, doxepin, simple petrolatum after sitz bath
Pruritic Dermatoses: Pruritus vulvae
• vulva is a common site for pruritus of different causes
• counterpart of pruritus scroti
• most common causes:
• unspecified dermatitis (54%)
• lichen sclerosus (13%)
• chronic vulvovaginal candidiasis (10%)
• dysesthetic vulvodynia (9%)
• psoriasis (5%)
• Contact dermatitis
• sanitary pads, contraceptives, douche solutions, fragrance, preservatives, colophony, benzo- caine,
corticosteroids, and a partner’s condoms
• Urinary incontinence
• Lichen sclerosus
• may involve the vulva, resulting in pruritus and mucosal changes, including erosions and ulcerations, resorp-
tion of the labia minora, and atrophy.
• Tx: pulsed dosing of high-potency topical steroids ; topical tacrolimus or pimecrolimus
Pruritic Dermatoses: Pruritus pruritica (itchy
points)
• one or two intensely itchy spots in clinically normal skin
• followed by the appearance of seborrheic keratoses at exactly the
same site.
• Tx
• Curettage, cryosurgery, punch biopsy, botulinum toxin A injection
Pruritic Dermatoses: Aquagenic pruritus and aquadynia

• itching evoked by contact with water of any temperature

• severe, prickling discomfort within minutes of exposure to water
• Diagnosis: initial trial of “soaking and smearing,”
• Tx
• anti- histamines
• sodium bicarbonate dissolved in bath water
• propranolol, SSRIs, acetylsalicylic acid (ASA, aspirin),
• Pregabalin
• Montelukast
• NB UVB or PUVA phototherapy
Pruritic Dermatoses: Scalp pruritus
• Lack of excoriations, scaling, or erythema excludes inflammatory causes
• Most cases remain idiopathic
• Inflammatory causes:
• seborrheic der-mastitis
• Psoriasis
• dermatomyositis
• lichen simplex chronicus
• Tx
• topical tar shampoos
• salicylic acid shampoos
• corticosteroid topical gels, mousse, shampoos, and liquids
• Severe: intralesional injection of corticosteroid suspension
• Minocycline or oral antihistamines
Pruritic Dermatoses: Drug-induced pruritus
• Medications
• Opioids
• Chloroquine
• Other antimalarials – esp African Americans treated for malaria
• SSRI
• Drugs causing cholestatic liver disease
• Hydroxyethyl starch (HES) - volume expander, a substitute for human
plasma
Pruritic Dermatoses: Chronic pruritic
dermatoses of unknown cause
• Prurigo simplex
• chronic itchy idiopathic dermatosis
• Lesion: prurigo papule
• dome shaped, topped with a small vesicle
• vesicle is usually present only transiently because of its immediate removal by scratching
• crusted papule is more frequently seen
• Common sites: symmetric
• Trunk
• extensor surfaces of the extremities
• Tx
• topical corticosteroids
• oral anti- histamines
• Intralesional injection of triamcinolone will eradicate individual lesions
• Recalcitrant disease: UVB or PUVA therapy
Pruritic Dermatoses: Prurigo pigmentosa
• rare dermatosis of unknown cause
• sudden onset of erythematous papules or vesicles that leave reticulated
hyperpigmentation when they heal
• associated with weight loss, dieting, anorexia, diabetes, and ketonuria
• exacerbated by heat, sweating, and friction
• Common sites: upper back, nape, clavicular region, and chest
• Spared: mucus membranes
• Histo: neutrophils in dermal papillae and epidermis
• Tx:
• Minocycline, 100–200 mg/day
• Dapsone
• Alteration of diet
Pruritic Dermatoses: Lichen simplex chronicus
• circumscribed neurodermatitis
• results from long-term chronic rubbing and scratching
• Lichenification
• skin becoming thickened and leathery
• striae form a crisscross pattern producing a mosaic in between composed of flat-topped, shiny, smooth
quadrilateral facets
• may originate on seemingly normal skin or may develop on skin that is the site of another disease
(atopic or allergic contact dermatitis)
• onset of this dermatosis is usually gradual and insidi- ous.
• Tx
• void scratching
• high-potency steroid cream or ointment (initially but not indefinitely)
• shifted to the use of medium- to lower- strength topical steroid creams as the lesions resolve.
• topical doxepin, capsaicin, or pimecrolimus cream or tacrolimus ointment provides significant antipruritic
effects and - good adjunctive therapy.
• intralesional injections of triamcinolone
Pruritic Dermatoses: Prurigo nodularis
• multiple itchy nodules mainly on the extremities
• anterior surfaces of the thighs and legs
• linear arrangement is common
• individual lesions are pea sized or larger, firm, and erythematous or brownish
• fully developed - verrucous or fissured.
• chronic, and the lesions evolve slowly
• Itching is severe, aggravated by stress
• characteristically paroxysmal: intermittent, unbearably severe, and relieved only by scratching to
the point of damaging the skin, usually inducing bleeding and often scarring.
• Tx:
• intralesional or topical administration of steroids.
• PUVA, NB UVB, and UVA a
• Vitamin D3 ointment, calcipotriene ointment, or tacrolimus ointment
• Isotretinoin
• thalidomide, lenalid- omide, pregabalin, and cyclosporine.
Psychodermatology
• cause being directly related to psychopathologic causes
• in the absence of primary dermatologic or other organic causes
• Major categories
• elusions of parasitosis
• psychogenic (neurotic) excoriations
• factitial dermatitis
• trichotillomania
• Monosymptomatic hypochondria- cal disorder
• No other mental deficits (audi- tory hallucination, loss of interpersonal skills, or
presence of other inappropriate actions
• monosymptomatic hypochondriacal psychosis often function appropriately in
social settings, except for a single fixated belief that there is a serious problem
with their skin or with other parts of their body.
Psychodermatology: Signs
• skin is a frequent target for the release of emotional tension
• Self- injury by prolonged
• may produce various mutilations
• Self-biting (Dermatophagia)
• may be manifested by biting the nails (onycho- phagia), skin (forearms, hands, fingers), and lip
• Bumping of head - produces lacerations and contusions
• Compulsive repetitive handwashing may produce an irritant dermatitis of the hands
• Bulimia, with its self-induced vomiting, results in Russell’s sign
• crusted papules on the dorsum of the dominant hand from cuts by the teeth.
• Clenching of the hand produces swell- ing and ecchymosis of the fingertips and subungual hemor-rhage
• Self-inflicted lacerations may be of suicidal intent
• Lip licking produces increased salivation and thickening of the lips.
• perioral area becomes red and produces a distinctive picture resembling the exaggerated mouth makeup of a clown
• Pressure produced by binding the waistline tightly with a cord will eventually lead to atrophy of the subcutaneous tissue.
• Tx
• Psychopharmacologic agents, especially the newer atypical antipsychotic agents,
• behavioral therapy alone
• or in com- bination with these agents
Psychodermatology: Delusions of parasitosis
• firm fixations in a person’s mind that he or she suffers from a parasitic infestation of the skin
• At times, close contacts may share the delusion
• he patient may pick small pieces of epithelial debris from the skin and bring them to be examined
• the only symptom is pruritus or a stinging, biting, or crawling sensation
• May have paranoid tendencies (W:M 2:1)
• associated with schizophre- nia, bipolar disorders, depression, anxiety disorders, and obsessional states
• Consider: cocaine, alcohol, and amphetamine abuse; dementia or other neurologic conditions
• malignancies, particularly lymphoma and leukemia; cerebrovascular disease; endocrine disorders; infectious diseases;
pellagra; and vitamin B12 deficiency
• Consider: meds: gabapentin, antiparkinsonian and antihistaminic drugs, and corticoste- roids
• Tx
• Refer to psych
• Develop trust, do not confront
• Pimozide (WOF SE tiffness, restlessness, prolongation of Q-T interval, and extrapyramidal signs)
Psychogenic (neurotic) excoriations
• unconscious compulsive habits of picking at themselves, and at times the tendency is so
persistent and pronounced that excoriations of the skin result
• patients admit their actions induce the lesions but cannot control their behavior.
• excavations may be superficial or deep and are often linear
• bases of the ulcers are clean or covered with a scab
• Right-handed persons tend to produce lesions on their left side and left-handed persons on their
right side.
• evidence of past healed lesions, usually with linear scars, or rounded hyperpigmented or hypopigmented
lesions
• Tx
• Doxepin (antidepressant and antipruritic effects)
• clomipramine, paroxetine, fluoxetine, and sertraline.
• Training in diversion strategies from scratching
• systematic training directed at the behavioral reaction pattern
• support and advice given with regard to the patient’s social situation and interpersonal relations.
Factitious dermatitis and dermatitis artefacta
• Factitious dermatitis
• self-inflicted skin lesions with the intent to elicit sympathy, escape responsibil- ity, or collect disability insurance.
• * Malingering – with intent of material gain
• Dermatitis artefacta
• unconscious goal of gaining attention and assuming the sick patient role.
• vast majority have multiple lesions and are unemployed or on sick leave.
• skin lesions are provoked by mechanical means or by the application or injection of chemical irritants and caustics
• have a “hollow” history, unable to detail how the lesions appeared or evolved
• usually have a distinctive, geo- metric, bizarre appearance
• generally distributed on parts easily reached by the hands, tend to be linear and arranged regularly and symmetrically
• Subset of px have Munchausen syndrome
• Tx
• psychotherapy - patient promptly rejects the suggestion
• provide symptomatic therapy and nonjudgmental support.
• SSRIs may address associated depression and anxiety
• Very-low-dose atypical antipsychotics
• Consultation with an experienced psychiatrist is prudent.
Trichotillomania
• neurosis characterized by an abnormal urge to pull out the hair.
• frontal region of the scalp, eyebrows, eyelashes, and the beard.
• irregular areas of hair loss, which may be linear or bizarrely shaped.
• Hairs are broken and show differences in length
• pulled hair may be ingested, and occasionally the trichobezoar will cause obstruction
• When the tail extends from the main mass in the stomach to the small or large intestine, Rapunzel syndrome is the diagnosis
• often develops in the setting of psychoso- cial stress
• nails may show evi- dence of onychophagy
• Diagnosis:
• Multiple catagen
• Shave part of involved area and observe for regrowth of normal hairs
• Tx
• In children, the diagnosis should be addressed openly, and referral to a child psychiatrist for cognitive-behavioral therapy
• Habit-reversal training
• In adults with the problem, psychiatric impairment may be severe
• clomip- ramine
• SSRIs
Dermatothlasia
• patient’s uncontrollable desire to rub or pinch themselves to form
bruised areas on the skin, sometimes as a defense against pain
elsewhere.
Bromidrosiphobia
• mono- symptomatic delusional state
• a person is convinced that his or her sweat has a repugnant odor that
keeps other people away
• unable to accept any evidence to the contrary.
Body dysmorphic disorder
• excessive preoccupation of having an ugly body part
• most common in young adults
• frequently centered about the nose, mouth, genitalia, breasts, or hair.
• Objective evaluation will reveal a normal appearance or slight defect
• Associated depression and social isolation along with other comorbidities
present a high risk of suicide
• Tx
• SSRI
• CBT
Cutaneous dysesthesia: Scalp dysesthesia
• pain and burning sensations without objective findings.
• middle-age to elderly women
• Associated with cervical spine degenerative disk disease
• chronic tension is placed on the occipitofrontalis muscle and scalp
aponeurosis
• Tx
• Gabapentin
• Antidepressants if with psychiatric overlay
Cutaneous dysesthesia: Burning mouth
syndrome (glossodynia, burning tongue)
• Primary
• burning sensation of the oral mucosa
• no dental or medical cause
• most frequently in postmenopausal women
• Tx: topical applications of clonazepam, capsaicin, doxepin, or lidocaine
• Oral administration of α-lipoic acid, SSRIs or tricyclic antidepressants (TCAs), gaba- pentin, and
benzodiazepines
• Secondary
• With cause: lichen planus, candidiasis, vitamin or nutritional deficiencies, hypoestrogenism,
parafunctional habits, dia- betes, dry mouth, contact allergies, cranial nerve injuries, and
medication side effects
• Tx: treat underlying dse
• Burning lips syndrome
• affect both men and women equally
• between ages 50 and 70.
• Tx: α-lipoic acid
Cutaneous dysesthesia: Vulvodynia
• vulvar discomfort, usually described as burning pain, occurring without medical findings.
• chronic, defined as lasting 3 months or longer.
• localized and generalized subsets.
• pain experienced may be debilitating. It may be accom- panied by pelvic floor abnormalities,
headaches, fibromyalgia, irritable bowel syndrome, and interstitial cystitis
• Tx
• patient and partner educa-ion and psychological support, including sex therapy and counseling
• Topical anesthetics and lubricants
• topical tacrolimus
• antihistamines
• Pelvic floor physical therapy
• CBT
• TCAs, SSRIs, and neuroleptics, chiefly gabapen- tin or pregabalin.
Notalgia paresthetica
• Unilateral sensory neuropathy
• characterized by infrascapular pruritus, burning pain, hyperalge- sia, and
tenderness
• often in the distribution of the second to sixth thoracic spinal nerves
• pigmented patch localized to the area of pruritus is often found
(postinflammatory change)
• degenerative changes in the corresponding vertebrae = spinal nerve
impingement
• Tx: physical therapy, nonste- roidal anti-inflammatory drugs (NSAIDs), gabapentin,
oxycar- bazepine, and muscle relaxants, paravertebral blocks
• Tx
• Topical capsaicin or lidocaine patch (WOF relapse)
Brachioradial pruritus
• localized to the bra- chioradial area of the arm
• To relieve the burning, stinging, or even painful quality of the itch, patients will frequently use ice packs
• majority will have the sun-induced variety,
• cervical spine pathology is frequently found on radiographic evaluation.
• Cause: spinal injury, such as trauma, arthritis, or chronic repetitive microtrauma, whiplash injury, or
assessment for a tumor in the cervical spinal column.
• Tx:
• Gabapentin, botulinum A toxin, topical amitriptyline- ketamine or capsaicin, aprepitant, carbamazepine
• cervical spine manipulation, neck traction
• anti-inflammatory medications
• physical therapy
• surgical resection of a cervical rib
Meralgia paresthetica (Roth-Bernhardt
disease)
• Characterized by
• Persistent numbness and periodic transient episodes of burning or lancinating pain on the anterolateral surface of the thigh
• lateral femoral cutaneous nerve
• subject to entrap- ment and compression along its course
• Alopecia localized to the area innervated (skin sign)
• Seen in
• middle-age obese men.
• diabetes mellitus is seven times more common
• Causes
• External compression (tight-fitting clothing, cell phones, or other heavy objects in the pockets or worn on belts, or seat belt injuries)
• Internal compression (arthritis of the lumbar vertebrae, a herniated disk, pregnancy, intra-abdominal disease)
• Diagnosis: somatosensory-evoked potentials of the lateral femoral cutaneous nerve.
• Tx:
• Local anesthetics (e.g., lidocaine patch)
• NSAIDs
• rest, avoidance of aggravating factors
• weight reduction
• Gsbapentin
• local infiltration with corticosteroids
• Surgical decom- pression of the lateral femoral cutaneous nerve – for px with intractable symptoms
Complex regional pain syndrome (CRPS)
• characterized by
• burning pain, hyperesthesia, and trophic disturbances resulting from injury to a peripheral nerve
• pain is disproportionate to the injury, which may have been a crush injury, laceration, fracture, hypothermia,
sprain, burn, or surgery
• Usualluy in upper and lower extremities
• skin of the involved extremity becomes shiny, cold, and atrophic and may perspire profusely.
• Other cutaneous manifestations:
• bullae, erosions, edema, telangiectases, hyperpigmen- tation, ulcerations, and brownish red patches with linear
fis- sures
• Diagnosis: Budapest diagnostic criteria, 3phase technetium bone scan
• Common complication: osetoporosis
• Tx
• Refer to neuro or anesth regarding pain
• Osteoporosis: pamidronate (inhibitor if bone absorption)
• Pain relief, physical and vocational rehabilitation, and psychological intervention
Trigemical trophie to c syndrome
• slowly enlarging, unilateral, uninflamed ulcer on ala nasi or adjacent cheek
skin due to interruption of the peripheral or central sensory pathways of
the trigeminal nerve
• Nasal tip is spared
• Cervical trophic syndrome: involvement of neck
• Secondary to herpes zoster-associated nerve injury
• Cause: self inflicted trauma to anesthetic skin
• Diagnosis:
• Biopsy (to exclude tumor or granulomatous or infectious etiologies)
• Tx
• Prevention by occlusion or with psychotropic medications
• Scarring may be severe
Mal perforans pedis
• neuropathic ulceration or perforating ulcer of the foot
• chronic ulcerative disease seen on the sole in conditions that result in loss of pain sensation at a site of constant trauma
• Primary cause
• posterolateral tracts of the cord (in arteriosclerosis and tabes dorsalis)
• lateral tracts (in syringomyelia)
• peripheral nerves (in diabetes or Hansen’s disease)
• begins as a circumscribed hyperkeratosis, usually on ball of foot
• Lesion
• becomes soft, moist, and malodorous and later exudes a thin, purulent discharge
• slough slowly develops, and an indo- lent necrotic ulcer is left that lasts indefinitely
• neuropathy renders the ulceration painless = plantar ulcers with thick callus
• Complication
• Osteomyelitis – deeper perforation and secondary infection
• Tx
• relief of pressure on the ulcer (total-contact cast)
• debridement of the surrounding callosity
• local and systemic antibiotics
Sciatic nerve injury
• an result from improperly per- formed injections into the buttocks.
• older patients are more susceptible
• decreased muscle mass
• presence of debilitating disease
• most common scenario: improper needle placement
• Other common causes
• hip surgery complications
• hip fracture and dislocation
• compression by benign and malignant tumors.
• Manifestations
• paralytic footdrop – most common finding
• sensory loss and absence of sweating over the distribution of the sciatic nerve branches.
• kin of the affected extremity becomes thin, shiny, and often edematous.
• TX
• surgical exploration, guided by nerve action potentials, with repair of the sciatic nerve
• Most successful if done soon after injury
Syringomyelia
• results from cystic cavities inside the cervical spinal cord caused by alterations of
cerebrospinal fluid flow
• Compression of the lateral spinal tracts produces sensory and trophic changes on the
upper extremities, particularly in the fingers
• Ssx
• gradually causes muscular weakness, hyperhidrosis, and sensory disturbances, especially in the
thumb and index and middle fingers
• skin changes are characterized by dissociated anesthesia with loss of pain and temperature sense
but retention of tactile sense.
• Burns are the most frequent lesions noted
• Bullae, warts, and trophic ulcerations occur on the fingers and hands, and eventually contractures
and gangrene occur
• Unusual features: hypertrophy of the limbs, hands, or feet, asymmetric scalp hair growth with a
sharp midline demarca- tion.
• Tx
• Early surgical tx
Hereditary sensory and autonomic
neuropathies
• Usually manifests with
• altered pain and temperature sensation, trophic changes, sweating
abnormalities, ulcers of the hands and feet, and in some patients, self-
mutilating behavior