VOHWINKEL SYNDROME

By Gary M. White, MD

The Honeycomb keratoderma of Vohwinkel Syndrome 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].

Clinical

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.

Treatment

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].

Additional Pictures

Pseudoainhum. Courtesy of Michael O. Murphy, MD
Vohwinkel Syndrome

References

Indian Journal of Dermatology 2014: 59: 619-621

RegionalDerm

Homepage | FAQs | Use of Images | Contact Dr. White


It is not the intention of RegionalDerm.com to provide specific medical advice, diagnosis or treatment. RegionalDerm.com only intends to provide users with information regarding various medical conditions for educational purposes and will not provide specific medical advice. Information on RegionalDerm.com is not intended as a substitute for seeking medical treatment and you should always seek the advice of a qualified healthcare provider for diagnosis and for answers to your individual questions. Information contained on RegionalDerm.com should never cause you to disregard professional medical advice or delay seeking treatment. If you live in the United States and believe you are having a medical emergency call 911 immediately.