title: Phacomatosis pigmentovascularis of cesioflammea type.
abstract: Phacomatosis pigmentovascularis is a rare syndrome, defined as the simultaneous presence of vascular nevus and melanocytic nevus in the same patient. We report the case of a 53-year-old woman presented with dermal melanosis and extensive vascular nevus, which match the typical manifestations of phakomatosis pigmetovascularis of cesioflammea type, according to Happle's classification. The rare occurrence of this genodermatosis and the clinical exuberance of the skin lesions motivated this case report.
authors.affiliation: Instituto de Dermatologia Professor Rubem David Azulay - Santa Casa da Misericórdia do Rio de Janeiro (IDPRDA - SCMRJ) - Rio de Janeiro (RJ), Brazil.
authors.forename: Delky Johanna Villarreal
authors.initials: DJ
authors.lastname: Villarreal
authors.affiliation: Instituto de Dermatologia Professor Rubem David Azulay - Santa Casa da Misericórdia do Rio de Janeiro (IDPRDA - SCMRJ) - Rio de Janeiro (RJ), Brazil.|Hospital Naval Marcílio Dias (HNMD) - Rio de Janeiro (RJ), Brazil.
authors.forename: Fabiano
authors.initials: F
authors.lastname: Leal
mesh_terms: D005260:Female; D006801:Humans; D008548:Melanosis / Q000473:pathology*; D008875:Middle Aged; D020752:Neurocutaneous Syndromes / Q000473:pathology*; D009508:Nevus, Pigmented / Q000473:pathology*; D019339:Port-Wine Stain / Q000473:pathology; D035583:Rare Diseases / Q000473:pathology; D012867:Skin / Q000473:pathology; D012878:Skin Neoplasms / Q000473:pathology*