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Andrews’ Diseases of the Skin-Chapter 10-pg 239-253 & Chapter 11. Boris Ioffe, D.O. Recalcitrant Palmoplantar Eruptions. Recalcitrant pustular eruptions of the hands and feet are often examples of psoriasis

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recalcitrant palmoplantar eruptions
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
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
dermatitis repens4
Dermatitis Repens
  • 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
dermatitis repens5
Dermatitis Repens
  • Histology
    • similar to those seen in psoriasis
    • the primary lesion is epidermal
    • An intraepithelial spongiform pustule is formed by infiltration of pmn’s
  • Treatment
    • topical mechlorethamine, topical steroids, PUVA, fluorouracil, and sulfapyridine
    • Acitretin, low dose cyclosporine, Acitretin plus calcipotriol
palmoplantar pustulosis
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
palmoplantar pustulosis7
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

Palmoplantar Pustulosis
palmoplantar pustulosis8
Palmoplantar Pustulosis
  • 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
palmoplantar pustulosis9
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

Palmoplantar Pustulosis
pustular bacterid
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
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
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
infantile acropustulosis16
Infantile Acropustulosis
  • 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
pompholyx
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
pomphylox
Pomphylox
  • Etiology- stress, atopy, and topical as well as ingested contactants
  • Histopathology: spongiotic vesicles in the epidermis
  • Differential dx:
    • 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
pomphylox20
Pomphylox
  • 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

Lamellar Dyshidrosis
lamellar dyshidrosis22
Lamellar Dyshidrosis
  • 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
Palmoplantar Keratoderma
  • AKA tylosis, keratosis, hyperkeratosis
  • Characterized by excessive formation of keratin on the palms and soles
  • Acquired
    • Keratosis Punctata of the Palmar Creases
    • Punctate Keratoses of the Palms and Soles
    • Porokeratosis Plantaris Discreta
    • Keratoderma Climactericum
  • Congenital
punctate keratosis of the palms and soles
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
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
slide28
Punctate keratoses of the palmar creases in an African-American

PPPK-punctate palmoplantar keratoderma

porokeratosis plantaris discreta
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
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
Hereditary syndromes
  • These have palmoplantar keratoderma as a feature
    • Unna-Thost
    • Papillon-Leferve
unna thost
Unna Thost
  • 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 thost33
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
papillon lefevre syndrome
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
papillon lefevre syndrome39
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
mutilating keratoderma of vohwinkel
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
slide43
Vohwinkel’s mutilating syndrome: A.) diffuse keratoderma of palms with B.) pseudoaainhum formation
palmoplantar keratodermas malignancy
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
acrokeratoelastoidosis of costa
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
exfoliative dermatitis
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%);

atopic dermatitis, chronic actinic dermatitis, psoriasis,seborrheic dermatitis, vesicular palmoplantar eczema, pityriasis rubra pilaris, and contact dermatitis

Drug eruptions(5%);

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%

Etiology
histology
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
treatment
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
parapsoriasis
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

Pityriasis Lichenoides Chronica
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

PLEVA
pleva65
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:

leukocytoclastic angiitis, papulonecrotic tuberculid, psoriasis, lichen planus, varicella, PR, drug eruptions, maculopapular syphilid, viral, rickettsial diseases, lymphomatoid papulosis

PLEVA
pleva67
PLEVA
  • 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
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
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

* Most lesions occur on the trunk and are between

1 –5cm

* Patches may persist for years to decades and do not progress to lymphoma

large plaque parapsoriasis
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
slide71
Large plaques parasporiasis: large, variably erythematous and mildly poikilodermatous patches in the bathing trunk region
treatment73
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
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
pityriasis alba76
Pityriasis Alba
  • 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
pityriasis alba77
Pityriasis Alba
  • 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
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
slide79
PR
  • Appears rapidly and last from 3-8 weeks
  • Peak: ages 15-40
  • Typically in Spring and Autumn
  • More common in women
pityriasis rosea80
Pityriasis Rosea
  • Mainly affects the trunk
  • Oral lesions are relatively uncommon, but present as aphthous lesions
pityriasis rosea82
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
treatment84
Treatment
  • Supportive
  • UVB should be used after acute inflammatory stage has passed
  • Topical corticosteroids
  • Antihistamines
  • Emollients
slide85
PR: There is focal parakeratosis, mild acanthosis, spongiosis, perivascular lymphocytes, and focal erythrocyte extravasation
slide88
PR in darkly pigmented skin: it tends to be more papular than in lightly pigmented skin-note associated hyperpigmentation
pityriasis rubra pilaris
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
slide90
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
slide91
PRP
  • 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
slide92
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
treatment94
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

Treatment
slide97
Pityriasis rubra pilaris: diffuse erythroderma with desquamation and follicular hyperkeratosis
slide98
Pityriasis rubra pilaris: follicular papules and confluent orange-red scaly plaques with islands of sparing