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Book

Vohwinkel Syndrome

Dahlia Saleh  1 , Haitham M. Saleh  2 , Laura S. Tanner
In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 Jan.
.
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Vohwinkel Syndrome

Dahlia Saleh et al.
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Excerpt

Vohwinkel syndrome, also known as keratoderma hereditarian mutilans, is classified as a type of hereditary palmoplantar keratoderma (PPK). PPKs exist on a spectrum from inherited to acquired and can range in their presentation. A PPK may be an isolated finding or part of a syndrome with extracutaneous involvement. Rarely a PPK can be drug-induced or part of a paraneoplastic process.

The classic Vohwinkel syndrome is a hereditary PPK associated with "starfish" keratoses on the knuckles, a PPK in a "honeycomb" pattern, hearing impairment, and mutilating digital constriction bands (pseudoainhum) that often lead to autoamputation of the affected digit(s).

A variant of Vohwinkel syndrome, loricrin keratoderma, presents as a honeycomb PPK with pseudoainhum with the addition of ichthyosis; deafness is not a feature of this ichthyosiform variant.

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Conflict of interest statement

Disclosure: Dahlia Saleh declares no relevant financial relationships with ineligible companies.

Disclosure: Haitham Saleh declares no relevant financial relationships with ineligible companies.

Disclosure: Laura Tanner declares no relevant financial relationships with ineligible companies.

References

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    1. Kallini JR, Sadeghani K, Khachemoune A. Paraneoplastic palmoplantar keratoderma secondary to metastatic uterine adenocarcinoma. Cutis. 2017 Mar;99(3):E32-E35. - PubMed
    1. Zhang M, Song K, Ding N, Shu C, Wang Y. Using a Distant Abdominal Skin Flap to Treat Digital Constriction Bands: A Case Report for Vohwinkel Syndrome. Medicine (Baltimore) 2016 Feb;95(6):e2762. - PMC - PubMed
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