Phacomatosis Pigmentovascularis Type II: Case Report

Phacomatosis pigmentovascularis is a rare syndrome first described by Ota et al, in 1947. It ischaracterized by a combination of capillary malformation and other pigmented naevi. It had originallybeen classified into four major types. A fifth type, in which the vascular lesion is cutis marmoratatelangiectatica congenita (CMTC), was subsequently added. Each type was further categorizedaccording to the absence or presence of associated extra-cutaneous signs as types (a) and (b),respectively. We reported this case due to its rare clinical presentation, with probable overlap Klippel-Trenaunay syndrome.