Variant of keratoderma hereditaria mutilans (Vohwinkel's syndrome). Treatment with orally administered isotretinoin

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Abstract
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. Three cases of a variant of KHM are described with an associated ichthyosiform dermatosis in a pedigree consisting of 19 affected individuals through 6 generations. An autosomal dominant inheritance pattern of KHM was confirmed. One of the patients was successfully treated with isotretinoin, 0.6 mg/kg per day orally. Five hypothetical genetic models to account for the simultaneous expression of palmar-plantar keratoderma and ichthyosiform dermatosis are presented.

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