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Journal of the American Academy of Dermatology
Volume 44, n° 2P2
pages 376-378 (février 2001)
Doi : 10.1067/mjd.2001.106348
Vohwinkel's syndrome in three generations
 

Renee R. Solis, BA, Dayna G. Diven, MD, Zoltan Trizna, MD, PhD
Department of Dermatology, University of Texas Medical Branch. Galveston, Texas 

Abstract

Vohwinkel's syndrome or keratoderma hereditaria mutilans is a diffuse, honeycombed, palmar, and plantar keratosis usually accompanied by pseudoainhum near the distal interphalangeal creases. The mutilating keratoderma associated with sensorineural hearing loss is thought to have an etiologic basis, resting on a mutation of the GJB2 gene, which encodes the gap junction protein connexin26 (Cx26). This specific mutation results in impaired epidermal differentiation as well as inner ear function. We describe a patient with Vohwinkel's syndrome accompanied by high-frequency sensorineural hearing loss whose mother and son were similarly affected. (J Am Acad Dermatol 2001;44:376-8.)

The full text of this article is available in PDF format.

 This supplement is made possible through an educational grant from Ortho Dermatological to the American Academy of Dermatology.
 No reprints available.
 J Am Acad Dermatol 2001;44:376-8



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