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 doença de Dowling-Degos é uma genodermatose rara, caracterizada principalmente por hiperpigmentação reticular progressiva de áreas flexurais. Apesar de apresentar evolução benigna, pode estar associada a neoplasias da pele. Além disso, as alterações cutâneas características potencialmente ocasionam prejuízo psicossocial, devido aos danos estéticos significativos. Os autores descrevem um caso dessa doença associado a ceratoacantoma e sintetizam os conceitos atuais sobre ela.
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