Vohwinkel syndrome: treatment of pseudo-ainhum.
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).


OBJECTIVE
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.


CONCLUSIONS
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.
Bassetto
Franco
F
Unit of Plastic Surgery, University of Padua, Padua, Italy.
Tiengo
Cesare
C
Sferrazza
Rossella
R
Belloni-Fortina
Anna
A
Alaibac
Mauro
M
D000387:Ainhum / Q000000981:diagnostic imaging / Q000209:etiology* / Q000601:surgery*; D000792:Angiography; D005260:Female; D005385:Fingers / Q000098:blood supply / Q000000981:diagnostic imaging / Q000601:surgery; D006801:Humans; D015776:Keratoderma, Palmoplantar, Diffuse / Q000150:complications* / Q000000981:diagnostic imaging / Q000601:surgery*; D008875:Middle Aged; D019651:Plastic Surgery Procedures; D013524:Surgical Flaps