Umschriebene angeborene vaskuläre malformation unter dem bild eines pseudo-Kaposi der haut: Langzeitbeobachtung über 8 Jahre

We are reporting the case of a 12-year old girl with at the time of first consultation painful, ulcerating pseudo-Kaposi based on an vascular malformation in the right ankle area. Although there were no direct signs of an arteriovenous fistula, a Stewart-Bluefarb syndrome was diagnosed, because of the clinical findings and the histology (convolutes of dilated, thinwalled, endothelial cell lined blood vessels). The pseudo-Kaposi tumours of the skin, which are typical for this syndrome, are benigne lesions resembling Kaposi's sarcoma. Usually the skin lesions appear unilateral on the lower limb in form of plaque-like, reddish-brown infiltrated tumours. Major clinical symptoms include oedema and pain as well as ulceration with a high risk of bleeding and infection. It is diagnosed after angiological and histological examinations. The curative therapy requires the elimination of the arteriovenous shunt by surgery or chemoembolisation. In the case presented here a two step excision of the entire lesion led to a very good long term result without any considerable recurrence.