A Case of Vohwinkel's Syndrome Succesfully Treated with Acitretin


Ceyhan A. M. , YILDIRIM M. , Akkaya V. B. , Calapoglu N. S.

TURKDERM-ARCHIVES OF THE TURKISH DERMATOLOGY AND VENEROLOGY, vol.43, no.4, pp.180-182, 2009 (Journal Indexed in SCI) identifier identifier

  • Publication Type: Article / Article
  • Volume: 43 Issue: 4
  • Publication Date: 2009
  • Title of Journal : TURKDERM-ARCHIVES OF THE TURKISH DERMATOLOGY AND VENEROLOGY
  • Page Numbers: pp.180-182

Abstract

Vohwinkel's syndrome (Keratoderma hereditaria mutilans) is a rare genodermatosis consisting of hyperkeratosis of the palms and soles with a characteristic "honeycomb" appearance, "starfish-shaped" keratotic plaques on the dorsum of hands and feet and fibrous constricting bands (pseudoainhum) at the interphalangeal joints Associated findings with the disease are cicatricial alopecia, ichthyosiform dermatoses, acoustic impairment, deafness, spastic paraplegia and myopathy, and nail anomalies Etretinate and isotretinoin have been used successfully in several patients with Vohwinkel's syndrome to reverse both the keratoderma and pseudoainhum in this report, we described a case with Vohwinkel's syndrome that presented with generalized ichthyosis, diffuse palmoplantar keratoderma, pseudoainhum, onychogryphosis, ectropion and bilateral sensorineural hearing loss and was successfully treated with acitretin, 35 mg/day orally. (Turkderm 2009; 43; 180-2)