RESUMO
Phacomatosis pigmentovascularis is a rare, congenital condition characterized by a combination of cutaneous melanocytic lesions and vascular malformation. We discuss an entirely unique case of Phacomatosis pigmentovascularis with nevus of Ota, extensive Mongolian spot, nevus flammeus, nevus anemicus and cutis marmorata telangiectatica congenita, which may represent a heretofore undescribed variant of phacomatosis pigmentovascularis.
Assuntos
Mancha Mongólica/patologia , Síndromes Neurocutâneas/patologia , Nevo de Ota/patologia , Mancha Vinho do Porto/patologia , Dermatopatias Vasculares/patologia , Neoplasias Cutâneas/patologia , Telangiectasia/congênito , Humanos , Livedo Reticular , Masculino , Síndromes Neurocutâneas/classificação , Telangiectasia/patologia , Adulto JovemRESUMO
Phacomatosis pigmentovascularis is a rare, congenital condition characterized by a combination of cutaneous melanocytic lesions and vascular malformation. We discuss an entirely unique case of Phacomatosis pigmentovascularis with nevus of Ota, extensive Mongolian spot, nevus flammeus, nevus anemicus and cutis marmorata telangiectatica congenita, which may represent a heretofore undescribed variant of phacomatosis pigmentovascularis.
.Assuntos
Humanos , Masculino , Adulto Jovem , Mancha Mongólica/patologia , Síndromes Neurocutâneas/patologia , Nevo de Ota/patologia , Mancha Vinho do Porto/patologia , Dermatopatias Vasculares/patologia , Neoplasias Cutâneas/patologia , Telangiectasia/congênito , Síndromes Neurocutâneas/classificação , Telangiectasia/patologiaRESUMO
Phacomatosis Pigmentovascularis is a rare syndrome characterized by capillary malformation and pigmentary nevus. A case of a 2-year-old patient is reported, who presented extensive nevus flammeus and an aberrant Mongolian spot, without systemic disease, manifestations that allow us to classify this case as type IIa Phacomatosis Pigmentovascularis, according to Hasegawa's classification.
Assuntos
Mancha Mongólica/patologia , Síndromes Neurocutâneas/patologia , Nevo de Ota/patologia , Neoplasias Cutâneas/patologia , Pré-Escolar , Doenças do Cabelo/patologia , Humanos , Masculino , Síndromes Neurocutâneas/classificação , Pele/patologiaRESUMO
Phacomatosis Pigmentovascularis is a rare syndrome characterized by capillary malformation and pigmentary nevus. A case of a 2-year-old patient is reported, who presented extensive nevus flammeus and an aberrant Mongolian spot, without systemic disease, manifestations that allow us to classify this case as type IIa Phacomatosis Pigmentovascularis, according to Hasegawa's classification.
A Facomatose Pigmentovascular, síndrome rara, é caracterizada pela presença concomitante de malformação capilar e nevos pigmentares. Relata-se o caso de um paciente de dois anos de idade com malformação capilar extensa e mancha mongólica aberrante sem comprometimento sistêmico, manifestações que o incluem no tipo IIa na classificação da Facomatose Pigmentovascular, segundo Hasegawa.