Phacomatosis pigmentovascularis type IIa - Case report * Facomatose pigmentovascular tipo IIa - Relato de caso

Abstract: Phacomatosis Pigmentovascularis is a rare syndrome characterized by capillary malformation and pig-mentary nevus. A case of a 2-year-old patient is reported, who presented extensive nevus flammeus and an aber-rant Mongolian spot, without systemic disease, manifestations that allow us to classify this case as type IIaPhacomatosis Pigmentovascularis, according to Hasegawa's classification.Keywords: Hemangioma; Nevus of Ota; Nevus, pigmented Resumo: A Facomatose Pigmentovascular, sindrome rara, e caracterizada pela presenca concomitante de malfor-macao capilar e nevos pigmentares. Relata-se o caso de um paciente de dois anos de idade com malformacaocapilar extensa e mancha mongolica aberrante sem comprometimento sistemico, manifestacoes que o incluem notipo IIa na classificacao da Facomatose Pigmentovascular, segundo Hasegawa.Palavras-chave: Hemangioma; Nevo pigmentado; Nevo de Ota Received on 03.11.2012.Approved by the Advisory Board and accepted for publication on 06.02.2013.* Work performed at Sanitary Dermatology Outpatient Clinic (ADS)- Secretariat of Health - Porto Alegre (RS), Brazil.Conflict of interest: NoneFinancial funding: None