Vohwinkel Syndrome

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.