Andrews’ Diseases of the Skin

Andrews’ Diseases of the Skin
By:Boris Ioffe, D.O.

Recalcitrant Palmoplantar Eruptions

* Recalcitrant pustular eruptions of the hands and feet are often examples of psoriasis
* Need to then search for lesions elsewhere on the body(e.g., scalp, ears, glans penis)
* Search also for a family history to confirm your suspicion

Dermatitis Repens
* Aka- acrodermatitis continua and acrodermatits perstans
* It’s a chronic inflammatory disease of hands and feet
* Rarely, can become generalized
* Usually, as a pustule or paronychia
* Occasionally, mucous membranes are involved
* Nails are often dystrophic or destroyed
* Lesions cause skin atrophy
* Crusted, eczematoid, and psoriasiform lesions may occur, and there may be moderate itching
* It is essentially unilateral in its beginning and asymmetrical throughout its entire course
* Histology
o similar to those seen in psoriasis
o the primary lesion is epidermal
o An intraepithelial spongiform pustule is formed by infiltration of pmn’s
* Treatment
o topical mechlorethamine, topical steroids, PUVA, fluorouracil, and sulfapyridine
o Acitretin, low dose cyclosporine, Acitretin plus calcipotriol

Palmoplantar Pustulosis
* AKA pustular psoriasis
* In contrast to dermatitis repens it is essentially bilateral and symmetrical
* Locations include: thenar/hypothenar eminences or central portion of the palms and soles
* Patches begin as erythematous areas in which pustules form
* Start as pinhead-sized, enlarge and coalesce to form small lakes of pus
* In the course of a week, they tend to dry up, leaving punctate brown scabs that eventually exfoliate
* Stages of quiescence and exacerbation characterize the condition
* Meds, such as lithium, have been reported to induce
* Nails may become malformed, ridged, stippled, pitted and discolored
* May be associated with psoriasis vulgaris
* Some regard palmoplantar pustulosis as a form of psoriasis, while others consider it a separate entity
* Female predominance; lack of seasonal variation; different histopathologic features and
* Associated with thyroid disorders and cigarette smoking
* May be predisposed to joint disease and possibly SAPHO syndrome-Synovitis, Acne, Pustulosis, Hyperostosis and Osteoarthritis
* It’s resistant to most treatments
* Acitretin is reportedly effective(1mg/kg/day)
* Low-dose cyclosporine (1.25mg/kg/day-3.75mg/kg/day)
* Intramuscular Kenalog (40-60mg)may be effective for short-term relief

Pustular Bacterid
* Characterized by a symmetric, grouped, vesicular or pustular eruption on palms and soles
* Marked by exacerbations and remissions over long periods
* No involvement of webs of fingers or toes or flexion creases of toes
* WBC may be elevated
* Scaling is usually present
* Etiology is thought to be a remote focus of infection; infection needs to be treated before resolution will occur

Juvenile Plantar Dermatosis
* Usually begins as a patchy, symmetrical, smooth, red, glazed macule on great toes, sometimes with fissuring and desquamation in children aged 3-13
* Toe webs are rarely involved; fingers may be
* Histologically, there is psoriasiform acanthosis and a sparse, lymphocytic infiltrate in the upper dermis
* Spongiosis is commonly present
* Tx: bed rest, cotton socks and topical steroids
* Spontaneous resolution within 4 yrs is the rule

Infantile Acropustulosis
* Intensely itchy vesicopustular eruption of hands and feet
* Begins at any age up to 10 months, clearing in a few weeks and recurring repeatedly until final resolution at 6 – 36 months of age
* Dapsone at 2mg/kg/day may help
* Potent topical steroids aid in symptomatic relief
* Should prompt an extensive workup to eliminate serious infectious causes (i.e., Tzanck prep, gram stain, KOH prep of pustule)
* Some suspect that this condition may be a persistent reaction to prior scabies

Infantile Acropustulosis
* Acropustulosis of infancy
Pompholyx
* AKA dyshidrosis
* A vesicular eruption of palms and soles characterized by spongiotic intraepidermal vesicles and often accompanied by burning or itching
* Hyperhidrosis may be present
* Usually bilateral and symmetrical
* Bullae may form
* Contents are clear and colorless
* Attacks generally last a few weeks
* Lesions dry-up and desquamate rather than rupture
* Etiology- stress, atopy, and topical as well as ingested contactants
* Histopathology: spongiotic vesicles in the epidermis
* Differential dx:
o dermatophytid, contact dermatitis, atopic dermatitis, drug eruption, pustular psoriasis of palms and soles, acrodermatitis continua, and pustular bacterid
* Rarely, T-cell lymphoma can present with similar clinical findings, but biopsy of the vesicles will be diagnostic
* Tx: high potency corticosteroid creams
* Triamcinolone acetonide intramuscularly or a short course of oral prednisone is rapidly effective
* Oral or topical psoralen + UVA (PUVA) is effective but costly & inconvenient
* In more severe forms, immunosuppressive mycophenolate mofetil has been effective

Lamellar Dyshidrosis
* AKA dyshidrosis lamellosa, keratolysis exfoliativa
* A superficial exfoliative dermatosis of the palms and sometimes soles
* Referred to as recurrent palmar peeling
* Involvement is bilateral
* Can occur in association with dyshidrosis
* Often exacerbated by environmental factors
* Differential dx: dermatophytosis, chronic contact dermatitis
* Tx: difficult
* Spontaneous involution can occur in a few weeks for some
* Most tends to be chronic and relapsing
* Tar creams (Zetone cream) usually helps
* 5% tar in gel (Estar Gel) is an excellent tx
* Lac-Hydrin lotion and Carmol 10 or 20 are often effective
* NB-UVB may be helpful

Palmoplantar Keratoderma
* AKA tylosis, keratosis, hyperkeratosis
* Characterized by excessive formation of keratin on the palms and soles
* Acquired
o Keratosis Punctata of the Palmar Creases
o Punctate Keratoses of the Palms and Soles
o Porokeratosis Plantaris Discreta
o Keratoderma Climactericum
* Congenital


Punctate Keratosis of the Palms and Soles
* Primary lesion is a 1-5mm round to oval, dome-shaped papule distributed over left hand and hypothenar eminence
* Main symptom is pruritis
* Lesions number from 1 to >40
* Affects mainly blacks
* There’s a potential risk of developing lung and colon cancer

Keratosis Punctata of the Palmar Creases
* Common most often in black pts
* Primary lesion is a 1-5mm depression filled with a conical keratinous plug
* Primarily, in creases of palms or fingers, occasionally in soles
* Lesions are multiple
* Friction aggravates lesions causing them to become verrucoid or surrounded by callus
* Punctate keratoses of the palmar creases in an African-American
* PPPK-punctate palmoplantar keratoderma

Porokeratosis Plantaris Discreta
* Occurs in adults, Female:Male (4:1)
* Characterized by sharply marginated, rubbery, wide-based papule that does not bleed on removal
* Lesions are multiple, painful, 7-10mm in diameter
* Usually on wt bearing areas of sole, beneath metatarsal heads
* Tx: foot pads to redistribute wt, surgical excision, blunt dissection

Keratoderma Climactericum
* Characterized by hyperkeratosis of palms and soles beginning at about the time of menopause
* Descrete, thickened, hyperkeratotic patches most pronounced at pressure sites
* Fissuring may be present
* Tx: keratolytics -- 10% salicylic acid, lactic acid creams, etc.

Hereditary syndromes
* These have palmoplantar keratoderma as a feature
o Unna-Thost
o Papillon-Leferve
* Dominant inheritance; congenital thickening of epidermal horny layer of the palms and soles
* Usually symmetrical
* Epidermis becomes thick, yellowish, verrucous, and horny
* Striate and punctate forms occur

Unna Thost
* Occasionally nails become thickened
* 5% salicylic acid may help
* Lac Hydrin 12% may be tried
* Acitretrin or isotretinoin may be considered, but need for lifetime tx makes them impractical
* Focal palmoplantar keratosis of the striate type on the sole
* Diffuse non-epidermolytic palmoplantar keratosis
* Diffuse epidermolytic palmoplantar keratosis with diffuse hyperkeratosis

Papillon-Lefevre Syndrome
* Palmoplantar hyperkeratosis with peridontosis
* Usually develops within the first few months of life but may occur in childhood
* Well demarcated, erythematous, hyperkeratotic lesions on palms and soles
* Transverse grooves of fingernails may occur
* Early onset peridontal disease has been attributed to damage and alteration in PMN function caused by Actinomyces actinomycetemcomitans
* Disease associations include: acroosteolysis, and pyogenic liver abcesses
* There are asymptomatic ectopic calcifications in the choroid plexus and tentorium
* Therapy may retard both dental and skin abnormalities
* Treatment with Acitretin in four siblings was reported to be effective

Papillon-Lefevre Syndrome
* Papillon-Lefevre syndrome: plantar keratoderma

Mutilating Keratoderma of Vohwinkel
* Palmoplantar hyperkeratosis of the honeycomb type-associated with starfish-like keratosis on backs of hands and feet; linear keratoses of the elbows and knees, and annular constriction (pseudo-ainhum) of the digits, this may progress to autoamputation
* More than 30 cases have been reported world-wide
* More common in women and in whites
* Onset is in infancy or early childhood
* Vohwinkel’s mutilating syndrome: A.) diffuse keratoderma of palms with B.) pseudoaainhum formation

Palmoplantar Keratodermas & Malignancy
* Diffuse, waxy keratoderma of palms and soles occurring as an AD trait associated with esophageal carcinoma
* Other related factors are oral leukoplakia, esophageal srictures, squamous carcinoma of tylotic skin, carcinoma of larynx and stomach
* Acquired forms of palmoplantar keratodermas have also been associated with carcinoma of esophagus, lung, breast, bladder and stomach
* Focal PPK in association with carcinoma of the esophagus

Acrokeratoelastoidosis of Costa
* AD, more common in women
* Small, round, firm papules occurring over dorsal hands, knuckles, and lateral margins of palms and soles
* Appears in early childhood and progress slowly
* Most often asymptomatic
* Significant histologic finding is dermal elastorrhexis
* Therapies: liquid nitrogen, salicylic acid, tretinoin, and prednisone have been tried
* Focal acrokeratoelastoides: multiple skin-colored papules at the margin of the palmar skin
* Path: non-epidermolytic palmoplantar keratosis, acanthosis and hypergranulosis

Exfoliative Dermatitis
* Universal or very extensive scaling and itching erythroderma
* Often associated with hair loss
* Initially with erythematous plaques, which spread rapidly
* Onset accompanied by general toxicity
* Skin becomes scarlet and swollen and may ooze a straw-colored exudate
* Desquamation is evident within a few days

Etiology
* Most common is preexisting dermatoses: (53%);
o atopic dermatitis, chronic actinic dermatitis, psoriasis,seborrheic dermatitis, vesicular palmoplantar eczema, pityriasis rubra pilaris, and contact dermatitis
* Drug eruptions(5%);
o allopurinol, gold, carbamazepine, phenytoin, and quinidine
* Cutaneous T-cell lymphoma(13%); Sezary syndrome and mycosis fungoides
* Paraneoplstic (2%); carcinoma of the lung and carcinoma of the stomach
* Leukemia cutis (1%)
* Idiopathic (26%)
* Mortality rate at a mean follow-up interval of 51 months was 43%
Histology
* Most commonly, histology is nonspecific
* Hyperkeratosis & focal parakeratosis
* Epidermis shows mild acanthosis, scant superficial upper dermal infiltrate of mononuclear cells
* May be small areas of spongiosis
* Generalization after withdrawal of methotrexate
* Exfoliation of scale with underlying erythema
* Generalized erythema with thick scale and crusted fissures on the plantar surface
Treatment
* Topical steroids, soaks, and compresses
* Acitretin and cyclosporin-useful in psoriatic erythroderma, and isotretinoin in erythroderma caused by RPR; methotrexate
* Systemic corticosteroids in severe cases
* Discontinuing the offending drug in drug-induced cases
* Subungual hyperkeratosis and distal dystrophy

Parapsoriasis, Pityriasis Rosea, Pityriasis Rubra Pilaris
Parapsoriasis
* Group of macular scaly eruptions with slow evolution
* These are all markedly chronic, resistant to treatment, and are without subjective symptoms
* They are divided into: pityriasis lichenoides chronica, pityriasis lichenoides et varioliformis acuta, and parapsoriasis en plaques

Pityriasis Lichenoides Chronica
* Erythematous, yellowish, scaly macules and lichenoid papules
* They persist indefinitely without change
* Mainly on sides of trunk, thighs, and upper arms
* May be confused with psoriasis and secondary syphilis
* Tx- UV light is beneficial; however intense doses may be needed for good results
* PUVA has been reported to be effective
* Oral tetracycline may be used with antihistamines
* PLC is a benign disease that clears spontaneously in a few yrs to months
PLEVA
* AKA: parasoriasis lichenoides, Habermann’s disease, Mucha-Habermann disease and parapsoriasis varioliformis acuta
* Sudden appearance of a polymorphous eruption composed of macules, papules, and occasional vesicles
* May run an acute, subacute, or chronic course
* Papules are usually yellowish or brownish-red, round lesions, which tend to crust, become necrotic and hemorrhage
* When exanthem heals it leaves a smooth, pigmented, depressed, varioliform scar
* Favorite sites are anterior trunk, flexural arms, and axillae
* Palms and soles are involved infrequently-mucous membranes are not
* Generalized lymphadenopathy can occur
* Usually a benign, self-limited disorder, but may be more chronic and severe
* Maybe a spectrum of cutaneous T-cell lymophoma
* Differential dx:
o leukocytoclastic angiitis, papulonecrotic tuberculid, psoriasis, lichen planus, varicella, PR, drug eruptions, maculopapular syphilid, viral, rickettsial diseases, lymphomatoid papulosis
* Histologically of PLEVA is characterized by epidermal necrosis, with prominent hemorrhage and primarily a dense perivascular infiltrate of lymphocytes in the superficial dermis
* Absence of neutrophils simplifies the distinction between leukocytoclastic angiitis
* Lymphomatoid papulosis differs by the presence of large, atypical mononuclear cells in the dermal infiltrate

PLEVA-Tx
* No one tx is reliably effective
* Tetracycline and erythromycin are worth trying
* UVB and PUVA
* Methotrexate, 2.5-7.5mg every 12 hrs for 3 doses 1 day each week
* Several serious reactions a few of them fatal have occurred with simultaneous administration of methotrexate and NSAIDs
* Dapsone and pentoxifylline(Trental), 400mg twice daily


Parapsoriasis en Plaques
* Small-plaque parapsoriasis is characterized by non-indurated, brownish, hypopigmented, or yellowish red scaling patches, round to oval, with sharply defined borders
Large Plaque Parapsoriasis
* Has patches 5-15 cm; otherwise is similar to small-plaque type
* Prognosis is benign, especially if pruritis is severe
* 10% may eventuate in T-cell lymphoma
* Large plaques parasporiasis: large, variably erythematous and mildly poikilodermatous patches in the bathing trunk region
* Small plaque parasporiasis: small(<5), erythematous, slightly scaly patches
Treatment
* First line: UV radiation -- either natural or UVB
* Lubricants and Topical steroids
* PUVA but only if UVB fails
* Use of PUVA or high-potency topical streroids should be limited due to long-term adverse effects
* LPPP has the potential to develop lymphoma – thus, justifying more intense tx
* Vitamin D2 daily–250,000 units over 2-4 months has been effective

Pityriasis Alba
* AKA-pityriasis streptogenes, furfuraceous impetigo, pityriasis simplex, pityriasis sicca faciei, and erthema streptogenes
* Characterized by hypopigmented, round to oval, scaling patches on face, upper arms, neck, or shoulders
* Color is white (but never actually depigmented) or light pink
* Scales are fine and adherent
* Patches are usually sharply demarcated; edges may be erythematous and slightly elevated
* Lack of any early specifically follicular localization helps to distinguish this lesion from follicular mucinosis
* Vellus hairs are not lost in pityriasis alba, nor does hypesthesia to cold occur, as often happens in follicular mucinosis
* Usually asymptomatic; however there may be mild pruritis
* Disease mainly occurs in children and teenagers
* It is particularly a cosmetic problem in dark-skinned individuals
* Etiology unknown
* Excessively dry skin appears to be contributory
* Most lesions disappear with time
* Repigmentation can be accelerated with treatment
* Emollients and bland lubricants
* Low-strength corticosteroids plus Lac-Hydrin are helpful
* Others have recommended PUVA

Pityriasis Rosea
* Mild inflammatory exanthem of unknown origin ?viral
* Characterized by salmon-colored papules and patches which are oval and covered with a collarette of scale
* Disease frequently begins with a single herald patch, which may persist a week or more, then involutes
* Appears rapidly and last from 3-8 weeks
* Peak: ages 15-40
* Typically in Spring and Autumn
* More common in women
* Mainly affects the trunk
* Oral lesions are relatively uncommon, but present as aphthous lesions

Herald Patch
Pityriasis Rosea
* Papular PR is an unusual form common in black chidren under age 5
* Inverse PR is unusual, but not rare
* Relapses and recurrences are frequently observed
* A PR-like eruption can occur as a rxn to captopril, arsenicals, gold, bismuth, clonidine, methoxypromazine, tripelennaminehydrochloride, or barbituates
* Inverse pityriasis rosea: oval annular plaques in groin

Treatment
* Supportive
* UVB should be used after acute inflammatory stage has passed
* Topical corticosteroids
* Antihistamines
* Emollients
* PR: There is focal parakeratosis, mild acanthosis, spongiosis, perivascular lymphocytes, and focal erythrocyte extravasation
* PR: papules and annular plaques
* PR: oval and round plaques, some with central scale and others with a collarette of scale
* PR in darkly pigmented skin: it tends to be more papular than in lightly pigmented skin-note associated hyperpigmentation

Pityriasis Rubra Pilaris
* Chronic skin disease characterized by small follicular papules, disseminated yellowish pink scaling patches, and often, solid confluent palmoplantar hyperkeratosis
* Disease generally manifests itself first by scaliness and erythema of the scalp

PRP
* Involvement is usually symmetrical and diffuse, with islands of normal
* Hyperkeratosis of palms and soles called, the “sandal”
* Nails may be dull, rough, thick, and brittle
* Itching in some cases
* Koebner’s phenomenon may be present
* A number of cases have been associated with Kaposi’s sarcoma, leukemia, basal cell, lung, unknown primary metastatic and hepatocellular carcinoma
* PRP may classified into familal or acquired types

in respect to the onset of the disease in childhood or adulthood

* Griffth’s classification: Type I, the classic adult type, is seen most commonly, with 80% involuting in 3 years
* Three types of juvenile-onset forms account for up to 40% of cases and have a poor prognosis for involution
* Etiology unknown-??AD
* Either sex affected
* Possible related to deficiency of

vitamin A
* Histology: hyperkeratosis, follicular plugging, and focal parakeratosis at follicular orifice
* Inflammatory infiltrate in dermis is composed of mononuclear cells
* PRP: psoriasiform dermatitis with follicular plugging
Treatment
* Symptomatic: emollients-- Lac-Hydrin
* A several-month course of isotretinoin in doses of 0.5 – 2 mg/kg/day
* Vitamin A in doses of 300,00 to 500,000 untis daily, with possible addtion of vitamin E, 400 units 2-3 times daily
* Methotrexate 2.5mg alternating with 5mg daily
* Monitor and treat secondary infections
* Pityriasis rubra pilaris: diffuse erythroderma with desquamation and follicular hyperkeratosis
* Pityriasis rubra pilaris: follicular papules and confluent orange-red scaly plaques with islands of sparing
* Pityriasis rubra pilaris: orange-red waxy keratoderma of the palms

Andrews’ Diseases of the Skin.ppt