Document Detail


Vohwinkel syndrome: treatment of pseudo-ainhum.
MedLine Citation:
PMID:  20465619     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
BACKGROUND: Vohwinkel syndrome or keratoderma hereditaria mutilans is a rare autosomal dominant palmoplantar keratosis which manifests in infants and becomes more evident in adulthood. Patients with this mutation present hyperkeratosis of the palms and soles, constricting bands of the digits, usually at the fifth, and starfish-shaped hyperkeratosis on the dorsal aspects of the hands and feet. The disease mostly occurs in white women, where constricting fibrous bands appear on the digits and can lead to progressive strangulation and auto-amputation (pseudo-ainhum). AIM: The treatment of this keratoderma is very difficult and tends to be symptomatic: topical keratolytics and systemic retinoids have been used to treat hyperkeratosis, but without consistent results. Reconstructive surgery is utilized for the treatment of pseudo-ainhum. RESULTS AND CONCLUSION: In this study, we present an additional case of Vohwinkel syndrome in which constrictive bands of the fifth digit in the left hand were treated with a cross finger flap, with a favorable outcome after 18 months of follow-up.
Authors:
Franco Bassetto; Cesare Tiengo; Rossella Sferrazza; Anna Belloni-Fortina; Mauro Alaibac
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  International journal of dermatology     Volume:  49     ISSN:  1365-4632     ISO Abbreviation:  Int. J. Dermatol.     Publication Date:  2010 Jan 
Date Detail:
Created Date:  2010-05-14     Completed Date:  2010-08-20     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0243704     Medline TA:  Int J Dermatol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  79-82     Citation Subset:  IM    
Affiliation:
Unit of Plastic Surgery, University of Padua, Padua, Italy.
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MeSH Terms
Descriptor/Qualifier:
Ainhum / etiology*,  radiography,  surgery*
Angiography
Female
Fingers / blood supply,  radiography,  surgery
Humans
Keratoderma, Palmoplantar, Diffuse / complications*,  radiography,  surgery*
Middle Aged
Reconstructive Surgical Procedures*
Surgical Flaps

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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