Phacomatosis Pigmentovascularis (PPV) is a rare sporadic genodermatosis characterized by the combination of an extensive pigmentary nevus with a widespread vascular abnormality. PPV had originally been classified into four types and a fifth type was added later. Each type of PPV has two subtypes with subtype ‘a’ for cutaneous involvement only and subtype ‘b’ for cutaneous and systemic involvement. Here in we describe a case of PPV (type 2b) which presented with sturge-weber syndrome, widespread dermal melanocytosis, klippel-trenaunay syndrome. To the best of our knowledge, very few cases with these associations have been reported to date. Our case had widespread dermal melanocytosis (nearly most of the body), which made it unique.
Published Date: 2023-12-06; Received Date: 2023-11-08
