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, cilt.43, ss.180-182, 2009 (SCI İndekslerine Giren Dergi) identifier identifier

  • Cilt numarası: 43 Konu: 4
  • Basım Tarihi: 2009
  • Dergi Adı: TURKDERM-ARCHIVES OF THE TURKISH DERMATOLOGY AND VENEROLOGY
  • Sayfa Sayısı: ss.180-182

Özet

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)