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Anais Brasileiros de Dermatologia

On-line version ISSN 1806-4841

Abstract

CAVALCANTE, Lúcia Isabel de Sá; HOLANDA, Érica de Magalhães; ALMEIDA, Thereza Lúcia Prata de  and  ACCIOLY-FILHO, José Wilson. Vohwinkel´s mutilating keratoderma: report of three familial cases. An. Bras. Dermatol. [online]. 2003, vol.78, n.3, pp. 311-318. ISSN 1806-4841.  http://dx.doi.org/10.1590/S0365-05962003000300007.

Keratoderma hereditarium mutilans, or Vohwinkel´s syndrome, is a rare cutaneous disease characterized by thickening of the palms, soles and dorsa of the hands and feet, and by ainhum-like constriction of the fingers. This paper reports a case of three family members, over a span of two generations, who were affected by this disorder. Sensorineural deafness was also observed in each patient. We discuss differential diagnosis and retinoid treatment of this condition. This is the first case report on this disease to come from Brazil.

Keywords : ainhum; keratoderma; palmoplantar.

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