By Gary M. White, MD
Honeycomb keratoderma. Courtesy of Michael O. Murphy, MD
Vohwinkel Syndrome, also known as keratoderma hereditaria mutilans, is characterized by onset at three to four months of age of pseudoainhum followed by the development of honeycomb keratoderma and starfish keratosis on the dorsa of the hands and knees. Deafness is also a feature. Isolated cases and AD inheritance have both been reported. The cause appears to be a missense mutation in the gene that codes for connexin 26 [Hum Mol Genet 1999;8;1237]. A related entity is loricrin keratoderma which is characterized by pseudoainhum and palmoplantar keratoderma, but not deafness [BJD 2001;145;657].
This disease is characterized by the onset at three to four months of age of pseudoainhum followed by honeycomb keratoderma and starfish keratosis on the dorsa of the hands and knees.
Oral retinoids (e.g. acitretin or isotretinoin) may be tried. The combination of acitretin and topical adapalene was successful in one patient [JEADV 2016;30;169].
Pseudoainhum. Courtesy of Michael O. Murphy, MD
Indian Journal of Dermatology 2014: 59: 619-621
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