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Like epidermolytic hyperkeratosis, this disorder describes both a group of diseases and a histologic reaction pattern. The hallmark is suprabasilar clefting with acantholytic and dyskeratotic cells through all levels of the epidermis. Although sometimes grouped with blistering disorders, it is not usually associated with a clinical blister.


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GENERALIZED Darier's disease
GENODERMATOSIS Hailey-Hailey disease
INCIDENTAL Focal acantholytic dyskeratosis
MUCSOAL Vulval and anal acantholytic dyskeratosis

Focal acantholytic dyskeratosis of the anal canal.

Warkel RL, Jager RM.

Am J Dermatopathol 1986 Aug;8(4):362-3

Another case of focal acantholytic dyskeratosis of the anal canal.

Grossin M, Battin-Bertho R, Belaich S.

Am J Dermatopathol 1993 Apr;15(2):194-5

Acantholytic dyskeratotic epidermal naevus localized unilaterally in the cutaneous and genital areas.

Cottoni F, Masala MV, Cossu S.

Institute of Dermatology, University of Sassari, Italy.

Br J Dermatol 1998 May;138(5):875-8 Abstract quote

A 38-year-old woman presented with unilateral lesions on the left side of the body and in the genital area. Clinically, the lesions showed a polymorphic pattern: brownish papules in the axilla, keratotic comedo-like papules on the hand and foot, and whitish papular plaques on the labia majora and anal canal. There was no family history of skin diseases.

Histologically, cutaneous and mucosal specimens were characterized by acantholytic and dyskeratotic cells, corps ronds and grains in the parakeratotic zone, and by hyperkeratosis and parakeratosis. A diagnosis of epidermal naevus with acantholytic dyskeratosis was made.

Benign persistent papular acantholytic and dyskeratotic eruption of the vulva: a case report.

Pestereli HE, Karaveli S, Oztekin S, Zorlu G.

Department of Pathology, Akdeniz University, School of Medicine, Antalya, Turkey.

Int J Gynecol Pathol 2000 Oct;19(4):374-6 Abstract quote

We report a case of 44-year-old woman with persistent pruritic papules on the left and right labium majus of the vulva.

Histopathologic examination of the vulvar biopsy specimen revealed a suprabasal separation of the epidermis with acantholysis and dyskeratosis.


Vesicular lesions in a patient with lipoid proteinosis: a probable acantholytic dermatosis.

Ko C, Barr RJ.

Am J Dermatopathol. 2003 Aug;25(4):335-7 Abstract quote

Lipoid proteinosis is a rare genodermatosis with characteristic clinical and histologic findings later in life. We present a case of lipoid proteinosis in which the diagnosis was originally missed because the patient presented in childhood with vesicles and a hoarse voice and lacked any other manifestations. Microscopic examination of a vesicle revealed extensive non-dyskeratotic acantholysis.

Vesiculo-bullous lesions have been described clinically in lipoid proteinosis, but we are unaware of any histopathologic description regarding bullae formation. Although only a single patient is herein described, it seems unlikely that this type of acantholysis is purely coincidental.

It is proposed that lipoid proteinosis should be in the differential of acantholytic vesiculo-bullous lesions in a child.

PALMOPLANTAR Keratoderma variant
SOLAR-KERATOSIS RELATED Acantholytic actinic keratosis
SOLITARY Warty dyskeratoma
LINEAR Zosteriform Darier's disease or linear epidermal nevus
TRANSIENT Grover's disease

Henry JB. Clinical Diagnosis and Management by Laboratory Methods. Twentieth Edition. WB Saunders. 2001.
Rosai J. Ackerman's Surgical Pathology. Ninth Edition. Mosby 2004.
Sternberg S. Diagnostic Surgical Pathology. Fourth Edition. Lipincott Williams and Wilkins 2004.
Robbins Pathologic Basis of Disease. Seventh Edition. WB Saunders 2005.
DeMay RM. The Art and Science of Cytopathology. Volume 1 and 2. ASCP Press. 1996.
Weedon D. Weedon's Skin Pathology Second Edition. Churchill Livingstone. 2002
Fitzpatrick's Dermatology in General Medicine. 5th Edition. McGraw-Hill. 1999.
Weiss SW and Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. Fourth Edition. Mosby 2001.

Commonly Used Terms

Corp ronds and corp grains-Characteristic acantholytic changes occurring within the upper malpighian layer and stratum corneum respectively. Corp ronds are larger and subcorneal while corp grains resemble parakeratotic cells.

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Last Updated 7/28/2003

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