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Phacomatosis pigmentovascularis of cesioflammea type* * Work performed at the Instituto de Dermatologia Professor Rubem David Azulay da Santa Casa da Misericórdia do Rio de Janeiro (IDPRDA – SCMRJ) – Rio de Janeiro (RJ), Brazil.

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.

Keywords:
Melanocytes; Nevus; Nevus of Ota; Port-wine stain; Skin abnormalities; Skin pigmentation

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