What's the resolutive surgery for pseudo-ainhum in Vohwinkel syndrome? A case report and review of the literature

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 (pseudoainhu...

Full description

Saved in:
Bibliographic Details
Published in:Orthopedic Reviews 2021-02, Vol.12 (4), p.8868-8868
Main Authors: Rovere, Giuseppe, Stramazzo, Leonardo, Cioffi, Alessio, Galvano, Nicolò, Pavan, Davide, Restuccia, Giuseppe, D'Arienzo, Antonio, Capanna, Rodolfo, Maccauro, Giulio, D'Arienzo, Michele, Camarda, Lawrence
Format: Article
Language:English
Subjects:
Alopecia
Amputation
Baldness
Case Report
Case reports
Conflicts of interest
Hearing loss
Mutation
Orthopedics
Pain
Pseudoainhum, Hand surgey, Genetic disease, Vohwinkel Syndrome
Surgery
Surgical techniques
Sutures
Citations: Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary: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.
ISSN:2035-8237
2035-8164
DOI:10.4081/or.2020.8868