DOI: 10.36347/sjmcr.2015.v03i11.023

Pages: 1085-1087

Phakomatosis pigmentovascularis (PPV) is a rare syndrome characterized by extensive vascular naevus with pigmentary naevus with or without systemic manifestations. PPV has been classified into 4 types with subtype “a” for cutaneous involvement only and subtype “b” for cutaneous and systemic involvement. A case of 52 year old is reported, who presented with extensive naevus flammeus and verrucous epidermal naevus with episodes of seizure as systemic complain. This manifestation allows us to classify this as a case of Type 1b Phakomatosis pigmentovascularis, according to Hasegawa's classification. This case is presented due to the rare nature of the disease.