Villarreal Delky Johanna Villarreal, Leal Fabiano
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.
An Bras Dermatol. 2016 Sep-Oct;91(5 suppl 1):54-56. doi: 10.1590/abd1806-4841.20164516.
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.
色素血管性斑痣性错构瘤病是一种罕见的综合征，定义为同一患者同时存在血管痣和黑素细胞痣。我们报告了一例53岁女性病例，该患者表现为皮肤黑素沉着和广泛的血管痣，根据哈普尔分类，这与火焰色型色素血管性斑痣性错构瘤病的典型表现相符。这种遗传性皮肤病的罕见发生以及皮肤病变的临床丰富性促使了本病例报告的撰写。
