Phakomatosis pigmento vascularis with bulbar melanosis and oral changes in an Indian male patient - A rare case report

Abstract

Phakomatosis Pigmento Vascularis (PPV) refers to a developmental abnormality characterized by concurrence of pigmented and capillary malformation with or without systemic manifestations. Abnormal development of melanocytic nevus cells and vasomotor neural cells arising from the neural crest has been attributed in the pathogenesis of PPV. It is classified into 5 major subtypes. Although there is no sex predilection, a preponderance of these cases has been noted in Japan. Through this rare case report, we describe the oral changes of PPV type II b in a 28 year old Indian male patient.

Keywords

• melanosis
• nevi
• nevus of ota
• phakomatosis
• sturge-weber syndrome