Korean J Dermatol.  1994 Jun;32(3):537-541.

Phakomatosis Pigmentovascularis Type Ia

• Chung J
• Lee WS
• Ahn SK

Abstract

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 subclsssified the disorder into eight types : type I, nevus flammeus and nevus pigmentosus et verrucosus, type II, nevus flamme is and dermal melanocytosis, type III nevus flammeus and nev.us 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 had been present for approximately 7 years. He had no concon ittant systemic disease. The histopathologic findings on brown papules and purple patches are compatible with epidermal nevus and nevus flammeus respectively. We herein report a case of phakomatosis pigmentovascularis type I a.

Keyword

Down Syndrome; Familial Eruptive Syringoma

MeSH Terms

Adult
Down Syndrome
Hemangioma
Humans
Knee
Leg
Male
Neurocutaneous Syndromes*
Nevus
Port-Wine Stain
Thigh