Keratoderma hereditarium mutilans (Vohwinkel's syndrome) associated with congenital deaf-mutism

Keratoderma hereditarium mutilans, or Vohwinkel's syndrome, is a rare cutaneous disorder which is characterized by thickening of the palms, soles and dorsa of the hands and feet, and by ainhum-like constrictions of the fingers. We report a clinically typical case of Vohwinkel's syndrome in a 28-year-old, deaf-mute, woman. The patient presented with keratotic palms and soles which had a 'honeycomb' appearance, starfish-like keratoses on the dorsa of the hands, and pseudoainhum of the digits. Osteoporotic changes were present distal to the constricting bands. Successful treatment with retinoids has been reported recently, and the hyperkeratosis and constricting bands in our patient improved on therapy with etretinate.