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September 1989

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

Author Affiliations

Department of Dermatology University Hospital Gloriastrasse 31 8091 Zurich, Switzerland

Arch Dermatol. 1989;125(9):1284-1285. doi:10.1001/archderm.1989.01670210122027

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

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