Phakomatosis pigmentovascularis type-IIIb with Klippel-Trenaunay-Weber syndrome.

Abstract

Phakomatosis pigmentovascularis is characterized by the simultaneous occurrence of vascular and melanocytic or epidermal nevi in the same patient with or without systemic involvement. We present a patient with phakomatosis pigmentovascularis type IIIb accompanied with nevus flammeus and mottled hypermelanic nevus and associated with Klippel-Trenaunay-Weber syndrome. The pathogenesis of phakomatosis pigmentovascularis is still controversial. It has been proposed that the combination of vascular and pigmentary anomalies arises because of a genetic concept called the twin-spot phenomenon. Phakomatosis pigmentovascularis type IIIb is very rare and our case is the first one from Turkey.