Oligosymptomatic Form of Klippel-Trenaunay-Weber Syndrome Associated With Giant Nevus Spilus

To the Editor.—  In 1947 Ota et al¹ defined a rare combination of cutaneous hemangioma and pigmentary nevus as phacomatosis pigmentovascularis. The disorder was first subdivided into two types: type I, consisting of nevus flammeus and nevus pigmentosus et verrucosus, and type II, representing the association of nevus flammeus and aberrant mongolian spots. In 1966, Toda² described the coexistence of nevus flammeus and nevus spilus as the third type. Finally, type IV was defined in 1979 by Hasegawa and Yasuhara³ as nevus flammeus, aberrant mongolian spots, nevus spilus, and nevus anemicus.The case presented herein is designated as type IIIb according to the modified classification of phacomatosis pigmentovascularis published by Hasegawa and Yasuhara⁴ in which each type is subdivided into the following: (1) localized, and (2) systemic.

Report of a Case.—  A 9-year-old girl was referred to the Department of Dermatology, Zurich (Switzerland) University Hospital, because