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Skin - nontumor
Blistering disorders
Transient acantholytic dermatosis
Author: Nat Pernick, M.D, PathologyOutlines.com, Inc.
Revised: 1 November 2009
Last major update: November 2009
Copyright: (c) 2002-2009, PathologyOutlines.com, Inc.
Definition
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● Transient edematous papules and vesicles on chest and back, possibly due to heat and sweating
● Initially characterized by Grover in 1970 (Arch Dermatol 1970;101:426)
Terminology
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● Also called Grover's or Grover disease
● Proposed term is persistent and recurrent acantholytic dermatosis, since often is not transient (Hautarzt 2000;51:244)
Epidemiology
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● Incidence of 0.1%
● 70% male, mean age 61 years
Sites
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● Usually begins on anterior chest, upper back or lower chest
● May become disseminated, but scalp, palms and soles are spared
Etiology
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● Unknown
● Often associated with heat, sweating or sunlight exposure (Mayo Clin Proc 1999;74:229, Dermatology 1999;198:410), although many patients present in winter (J Am Acad Dermatol 2006;55:263)
● Associated with atopic dermatitis or asteatotic eczema
Clinical features
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● Benign, but often persistent and difficult to manage
● High index of suspicion needed to make the diagnosis
● All patients have some pruritis, may be severe; amount of itching does not correlate with amount of lesions
● Lesions are red-brown, discrete papules that usually do not coalesce
● 8% have coexisting hematologic malignancies (J Clin Oncol 1994;12:1703)
● No systemic symptoms
Prognostic factors
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● Most cases resolve within months, but often persists for years
Case reports
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● 14 year old girl with bullous variant (Eur J Dermatol 2005;15:288)
● 60 year old man with pruritic eruption on his back and chest (American Family Physician 2006)
● 71 year old man with disease induced by cetuximab (anti-EGFR) for colon cancer (Dermatology 2006;213:37)
Treatment
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● Topical corticosteroids for inflammation and itching
● Menthol or pramoxine lotions for itching
● Retinoids (Vitamin A, 50,000 Units 3/day x 2 weeks, then daily x 12 weeks, OR isotretinoid 40 mg/day x 2-12 weeks
● For severely resistant cases, oral corticosteroids, UV-B, PUVA, methotrexate or grenz radiation may be effective (eMedicine)
● Possibly triple antibiotics (Cutis 2007;80:72)
● Avoid excess heat or sweating
Clinical images
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Various images Bullous variant
Micro description (Histopathology)
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● Spongiosis and focal acantholysis
● Usually superficial dermal, perivascular infiltrate of lymphocytes and histiocytes; eosinophils in 25%
● May have other coexisting dermatologic disorders (Arch Pathol Lab Med 2009;133:1490)
Patterns:
● Pemphigus vulgaris–like (47%): predominantly limited to suprabasal acantholysis, with basal keratinocytes attached to basement membrane forming a characteristic tombstone appearance
● Darier-like (18%): suprabasal acantholysis of keratinocytes with scattered apoptotic or dyskeratotic cells within various levels of the epidermis
● Spongiotic (9%): edema within epidermis, causing separation of keratinocytes and prominent intracellular bridges
● Pemphigus foliaceous–like (9%)
● Mixed (9%)
● Hailey-Hailey–like (8%): suprabasal acantholysis of all levels of the epidermis without significant dyskeratosis (“dilapidated brick wall” appearance)
Micro images
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Acantholytic cells within Prominent acantholysis; Chronic lesion resembles
suprabasal clefts resembles Hailey-Hailey solar keratosis
disease
Acantholytic dyskeratosis With corps ronds Pemphigus vulgaris–like pattern
in epidermis and grains of suprabasal acantholysis
Various images Spongiosis Spongiotic-like pattern
Negative stains
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● No specific pattern for direct immunofluorescence
Differential diagnosis (pathology)
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● Darier’s disease: virtually identical; greater tendency to involve follicles; no eosinophilic infiltrate; clinical correlation essential
● Pemphigus vulgaris: broad zones of suprabasilar acantholysis; mucosal involvement; involvement of adnexal structures; often abundant eosinophils; fishnet pattern of IgG and C3 on direct immunofluoresence
● Pemphigus foliaceus: broad zone of subcorneal and subgranular acantholysis; involvement of adnexal structures; often eosinophils; fishnet pattern of IgG and C3 on direct immunofluoresence
● Primary spongiotic dermatitis: involvement of entire epidermis; psoriasiform hyperplasia
Differential diagnosis (clinical)
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● Dermatitis herpetiformis: severe pruritis, vesicles on extensor surface
● Drug eruption: morbilliform papules on face, trunk, and extremities
● Folliculitis: erythematous papules on trunk, head, and neck
● Galli-Galli disease (acantholytic variant of Dowling-Degos disease): presents in similar manner, but has a focal reticulated pattern, affects hands, groin and lower extremities; histology shows elongation of rete ridges but no acantholysis (J Am Acad Dermatol 2008;58:299)
● Parasites: examine scraping under microscope for organisms
End of Skin - nontumor > Transient acantholytic dermatosis
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