Abstract

CADAVID, MANUELA; ZAPATA, FRANCHEZCA  and  VELASQUEZ, NATALIA. Pigmentovascularis phacomatosis associated to Klippel-Trenaunay syndrome and triangular alopecia. A case report. CES Med. [online]. 2012, vol.26, n.2, pp.229-239. ISSN 0120-8705.

ABSTRACT Phacomatosis pigmentovascularis is a disorder characterized by cutaneous vascular malformations associated with melanocytic nevi: up to 50 % of patients have systemic involvement. The traditional classification system identifies 5 categories numbered from I to V according to the presence of certain types of nevi, and it subdivides the categories into type a and b, depending on whether or not systemic involvement is present. More recently, Happle proposed a new classification based on clinical findings as follows: cesioflammea (Mongolian spot and nevus flammeus), spilorosea (telangiectatic nevus and nevus Spilus) and cesiomarmorata (Mongolian spot and congenital telangiectatic cutis marmorata), the first one being the most common one. We present a case of a 3 year old male patient who presented at birth with a capillary vascular malformation, an extensive nevus flammeus, a Mongolian spot, scleral melanosis and mammilated iris, a suprapubic congenital venous malformation and triangular alopecia; he was diagnosed with cesioflammea phacomatosis pigmentovascularis that was later associated with Klippel Trenaunay Syndrome

Keywords : Phacomatosis pigmentovascularis; Klippel Trenaunay Weber Syndrome; Vascular malformations; Case reports.

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