Nevi flammei affecting two contralateral quadrants and nevus depigmentosus: A new type of phacomatosis pigmentovascularis?

Abstract

Here, we report on a 64-year-old-woman with nevi flammei affecting two contralateral quadrants, venous insufficiency of the right leg, and asymmetry of the arms, in association with a linear nevus depigmentosus. This combination of vascular and pigmentary abnormalities may represent a novel type of phacomatosis pigmentovascularis caused by non-allelic twin-spotting.