Mutilating palmoplantar keratoderma successfully treated with etretinate

Abstract

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.