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October 1984

Variant of Keratoderma Hereditaria Mutilans (Vohwinkel's Syndrome): Treatment With Orally Administered Isotretinoin

Author Affiliations

From the Division of Dermatology, Department of Medicine (Dr Camisa), and the Department of Biochemistry (Dr Rossana), Ohio State University, Columbus.

Arch Dermatol. 1984;120(10):1323-1328. doi:10.1001/archderm.1984.01650460063021

• Keratoderma hereditaria mutilans (KHM), or Vohwinkel's syndrome, is a rare genodermatosis consisting of hyperkeratosis of the palms and soles with a characteristic "honeycomb" appearance, keratotic structures taking the shape of a starfish and/or knuckle pads on the dorsal surfaces of the hands, and constricting bands (pseudoainhum) encircling digits of the hands and feet. We describe three cases of a variant of KHM with an associated ichthyosiform dermatosis in a pedigree consisting of 19 affected individuals through six generations. An autosomal dominant inheritance pattern for KHM was confirmed. One of the patients was successfully treated with isotretinoin, 0.6 mg/kg/day orally. We offer five hypothetical genetic models to account for the simultaneous expression of palmar-plantar keratoderma and ichthyosiform dermatosis.

(Arch Dermatol 1984;120:1323-1328)

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