Mutilating palmoplantar keratoderma successfully treated with etretinate.

Three patients, a mother and her two sons, with mutilating palmoplantar keratoderma (Vohwinkel's syndrome) were successfully treated with oral etretinate (Tigason). All of them had keratotic constriction furrows of one or more digits (pseudo-ainhum) with threatening spontaneous amputation. Oral treatment with etretinate brought about disappearance of the pseudo-ainhums and normalization of the digital blood circulation. The hyperkeratotic skin became thinned and pliable with a dose-dependent trend towards redness and atrophy. Continued long term etretinate medication has made it possible for these patients to fulfill their social and occupational activities. All 3 patients had acoustic impairment of high-pitched tone perception.