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Article
August 1986

Epidermolysis Bullosa Herpetiformis With Mottled Pigmentation and an Unusual Punctate Keratoderma

Author Affiliations

From the Department of Pathology (Drs Medenica-Mojsilović, Fenske, and Espinoza) and the Division of Dermatology, Department of Internal Medicine (Drs Fenske and Espinoza), University of South Florida College of Medicine, Tampa; and the Dermatology Section, James A. Haley Veterans Administration Hospital, Tampa, Fla (Dr Fenske). Dr Medenica-Mojsilović is now with the Department of Dermatology, University of Belgrade, Yugoslavia.

Arch Dermatol. 1986;122(8):900-908. doi:10.1001/archderm.1986.01660200072019
Abstract

• We report the clinical and pathological features of an epidermolytic form of epidermolysis bullosa (EB) present in a family with six affected members that was transmitted in an autosomal dominant manner in four generations. The essential clinical features included generalized herpetiform blistering of the skin, mottled pigmentation and palmo-plantar hyperkeratosis, both punctate and diffuse. Biopsy material obtained from fresh blisters, clinically intact preblistering skin, hyperkeratotic areas, and skin with mottled pigmentation was examined by light and/or electron microscopy. In addition to reporting a heretofore undescribed association of EB herpetiformis with mottled pigmentation and punctate keratoderma, we report previously undescribed histologic changes in the areas of punctate hyperkeratosis. Specifically, the unique histologic findings consisted of the presence of dyskeratotic cells with clear cytoplasm at the cellular periphery, parakeratosis, and involvement of the intradermal portion of the sweat duct. The possibility that these findings represent a new type of epidermolytic EB, rather than a variant of other types of epidermolytic EB, particularly EB herpetiformis or EB with mottled pigmentation, is discussed.

(Arch Dermatol 1986;122:900-908)

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