Vohwinkel syndrome: treatment of pseudo-ainhum.

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).
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.
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.
AuthorsFranco Bassetto, Cesare Tiengo, Rossella Sferrazza, Anna Belloni-Fortina, Mauro Alaibac
JournalInternational journal of dermatology (Int J Dermatol) Vol. 49 Issue 1 Pg. 79-82 (Jan 2010) ISSN: 1365-4632 [Electronic] United States
PMID20465619 (Publication Type: Case Reports, Journal Article)
  • 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

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