A Case of Phakomatosis Pigmentovascularis

ABSTRACT

In 1947, Ota et al. first reported a case of phakomatosis pigmentovascularis and defined a subgroup of this disease having a congenital generalized hemangioma and pigmented lesions including a mongolian spot-like lesion, nevus pigmentosus and nevus of Ota. We experienced a case of phakomatosis pigmentovasularis type IIb in a 10-day old male baby, who since birth, had generalized nevus flammeus and blue spots, syndactyly between 2nd and 3rd toes in both feet, and dilated collecting system in left kidney.