KERATODERMA HEREDITARIUM MUTILANS (VOHWINKELS-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 constriction s of the fingers, We report a clinically typical case of Vohwinkel's s yndrome in a 28-year-old, deaf-mute, woman. The patient presented with keratotic palms and soles which had a 'honeycomb' appearance, starfis h-like keratoses on the dorsa of the hands, and pseudoainhum of the di gits. Osteoporotic changes were present distal to the constricting ban ds. Successful treatment with retinoids has been reported recently, an d the hyperkeratosis and constricting bands in our patient improved on therapy with etretinate.