Phacomatosis melanorosea without extracutaneous features: an unusual type of phacomatosis pigmentovascularis.

Phacomatosis pigmentovascularis (PPV) represents a group of different types of didymosis (twin spotting) characterized by the coexistence of a large pigmentary nevus such as a mongolian spot, café-au-lait macule or macular nevus spilus, and an extensive telangiectatic nevus, such as nevus flammeus or nevus roseus. We describe a third case of phacomatosis melanorosea and discuss the denotation of this neologism.