Open AccessWhat’s the resolutive surgery for pseudo-ainhum in Vohwinkel syndrome? A case report and review of the literature
Author(s) -
Giuseppe Rovere,
Leonardo Stramazzo,
Alessio Cioffi,
Nicolò Galvano,
D. Pavan,
Giuseppe Restuccia,
Antonio D’Arienzo,
Rodolfo Capanna,
Giulio Maccauro,
Michele D’Arienzo,
Lawrence Camarda
Publication year - 2021
Publication title -
orthopedic reviews
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.412
H-Index - 13
eISSN - 2035-8237
pISSN - 2035-8164
DOI - 10.4081/or.2020.8868
Subject(s) - medicine , keratoderma , numerical digit , dermatology , surgery , hyperkeratosis , mathematics , arithmetic
Vohwinkel Syndrome, also known as Keratoderma Hereditarium Mutilans, is an extremely rare dominant autosomal keratosis. It typically presents with “starfish” keratoses on the knuckles, palmoplantar keratoderma (PPK), hearing impairment and mutilating digital constriction bands (pseudoainhum) that cause strangulation, often leading to autoamputation of the affected digit. Both medical and surgical treatment haven’t shown to date consistent results, in the treatment of pseudoainhum. In this study we present the case of a woman with Vohwinkel syndrome who showed constriction bands causing ischemic changes of the 5th digit of the right hand for which she was treated with surgery. We also present a review of the literature for the management of this disease.