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RESEARCH PRODUCT
What’s the resolutive surgery for Pseudo-Ainhum in vohwinkel syndrome? A case report and review of the literature
Antonio D'arienzoGiuseppe RestucciaGiuseppe RovereAlessio CioffiN GalvanoLeonardo StramazzoLawrence CamardaMichele D'arienzoGiulio MaccauroRodolfo CapannaDavide Pavansubject
VOHWINKEL SYNDROMEOrthopedic surgerymedicine.medical_specialtyKeratosisbusiness.industryPseudoainhum Hand surgey Genetic disease Vohwinkel SyndromeGenetic diseaseKERATODERMA HEREDITARIUM MUTILANSCase Reportmedicine.diseasePseudo-ainhumNumerical digitSurgeryPalmoplantar keratodermamedicineHand surgeyOrthopedics and Sports MedicineVohwinkel SyndromeSurgical treatmentbusinessskin and connective tissue diseasesPseudoainhumAutoamputationRD701-811description
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.
| year | journal | country | edition | language |
|---|---|---|---|---|
| 2021-02-01 |