[Pseudo-ainhum in Vohwinkel disease. Keratoma hereditarium mutilans]

Abstract

An 11-year-old Turkish boy who has suffered from palmoplantar keratosis since his first year of life is presented. He is the only one of a large family to be affected. The diffuse keratosis extends to the back of the hands and feet and still has a progressive course. At the age of 6 he developed a symmetric high-tone acoustic impairment and at 10, an ainhum-like constricting band around the fifth digit of the left hand. This constellation of symptoms is highly characteristic for mutilating keratoma (Vohwinkel's disease), which is a rare disorder of keratinization. The majority of cases in the literature have had an autosomal dominant pattern of inheritance, although sporadic cases like this have also been reported as well. If constricting band proceeds to the point where spontaneous amputation seems imminent, a therapy with orally administered retinoids should be considered.