Phakomatosis pigmentovascularis type Va. Case report and literature review.

Abstract

Phakomatosis pigmentovascularis (PPV) is a rare congenital syndrome characterized by concomitant vascular malformation and melanocytic lesions. The disorder may manifest only on the skin or be accompanied by abnormalities of other organs. PPV is divided into five types; the most reported case is type II, while type V is uncommon and the subtype Va is rare.