Epidermolytic Hyperkeratosis, Constriction Band, Vohwinkel Syndrome, Keratoderma, Palmoplantar - DermAtlas

Contributor

Bernard Cohen, MD

Diagnosis

epidermolytic hyperkeratosis constriction band Vohwinkel syndrome keratoderma, palmoplantar

Body Site

total body leg ankle foot

Age

38 years

Pigmentation

light

Organization

Color

skin color red

Morphology

papulosquamous (bump, scale)

Pattern

generalized, disseminated symmetric

Comments

This 38-year-old woman had a diffuse eruption typical of epidermolytic hyperkeratosis beginning shortly after brith. During adolescence she began to develop constriction bands of her fingers resulting in both autoamputation and surgical ampitation in an attempt to preserve function. The cutaneous findings of epidermolytic hypperkeratosis and Vohwinkel syndrome overlap clinically and histologically.

Description

marked thickening of the epidermis, exaggerated at the skin creases, and severe keratoderma with constriction bands of the digits associated with digit loss

Image Added

11/4/2003 7:22:57

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PubMed Medline Plus Medscape

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