A Case of Vohwinkel’s Syndrome Succesfully Treated with Acitretin

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)
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.

Asitretin Tedavisine Oldukça İyi Yanıt Veren Vohwinkel’s Sendromu Olgusu

Vohwinkel’s sendromu (keratoderma herediterya mutilans), karakteristik bal peteği görünümünde diffüz palmoplantar hiperkeratoz, el ve ayakların dorsal yüzünde yıldız şeklinde keratotik plaklar ve interfalangial eklemlerde konstriktif fibröz bantlar (psödoainhum) ile karakterize oldukça nadir görülen genodermatozdur. Skatrisyel alopesi, iktiyoziform dermatoz, işitme azlığı, sağırlık, spastik parapleji, miyopati ve tırnak anomalileri eşlik edebilen diğer bulgulardır. Vohwinkel’s sendromlu birçok hastada psödoainhum ve keratodermanın tedavisinde etretinat ve isotretinoin ile başarılı sonuçlar alınmıştır. Bu makalede generalize iktiyozis, diffüz palmoplantar keratoderma, psödoainhum, onikogrifoz, ektropion ve bilateral sensörinöral işitme azlığı saptanan ve 35 mg/gün oral asitretin tedavisine oldukça iyi yanıt veren Vohwinkel’s sendromu olgusu sunulmaktadır. (Türkderm 2009; 43: 180-2)