What's the Resolutive Surgery for Pseudo-ainhum in Vohwinkel Syndrome? A Case Report and Review of the Literature

Overview

Journal Orthop Rev (Pavia)

Specialty Orthopedics

Date 2021 Feb 26

PMID 33633820

Citations 3

Authors

Giuseppe Rovere

Leonardo Stramazzo

Alessio Cioffi

Nicolo Galvano

Davide Pavan

Giuseppe Restuccia

Antonio DArienzo

Rodolfo Capanna

Giulio Maccauro

Michele DArienzo

Lawrence Camarda

Affiliations

Soon will be listed here.

Abstract

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.

Citing Articles

Palmoplantar keratoderma, pseudo-ainhum and knuckle pads in an African patient: A case report.

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PMID: 34949162 PMC: 8705100. DOI: 10.1186/s12891-021-04945-8.

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