A Neonatal Case of Phacomatosis Pigmentovascularis Type IIa.

cutaneous vascular and pigmentary lesions. The pregnancy of his mother and family history were unremarkable. Physical examination showed bilateral nevus flammeus involving the scalp, face, neck, trunk, upper part of the back, scrotum and the flexor sides of arms and thighs. Large, bluish-grey pigmentation was observed on the abdominal area, back, buttocks, legs and the extensor sides of the arms (Figure 1a, b). Laboratory examination, abdominal ultrasound imaging and magnetic resonance imaging of the brain were normal. Systemic involvement was not detected. Phacomatosis pigmentovascularis (PPV) type IIa was diagnosed. Phacomatosis pigmentovascularis is a rare disorder characterized by the coexistence of vascular malformations and pigmentary lesions of skin. The first description of the disease was reported by Ota et al. (1), and Hasegawa et al. (2) classified the disease into four types. Nevus flammeus was the constant vascular malformation for all types. The associated pigmentary lesions were nevus pigmentosus et verrucosus in type I, Mongolian spots with or without nevus anemicus in A Neonatal Case of Phacomatosis Pigmentovascularis Type IIa