Phakomatosis pigmentovascularis is characterized by the coexistence of pigmentary nevus and hemangioma. Since the term was first described by Ota et al in 1947, about 80 cases have been reported in the literature, in 1979, Hasegawa et al
subclassified
the disorder into eight types : type 1, nevus flammeus and nevus pigmentosus et verrucosus, type II, nevus flammeus and dermal melanocytosis, type III nevus flammeus and nevus spilus, type IV, nevus flammeus, dermal melanocytosis and nevus
spilus.
Each
type is subdivided into localized(a) and systemic(b) types.
A 25-year-old male had brown papules on the left knee and thigh and purple patches on the left leg that that had been present for approximately 7 years. He had no concomittant systemic disease. The histopathologic findings on brown papules and
purple
are compatible with epidermal nevus and nevus flammeus respectively.
We herein report a case of phakomatosis pigmentovascularis type 1 a. (Kor J Dermatol 1994 ; 32(3) : 537~541)
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