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[Combined naevus flammeus and naevus fuscocoeruleus: phacomatosis pigmentovascularis type IIa].

Authors
  • Achtelik, W
  • Tronnier, M
  • Wolff, H H
Type
Published Article
Journal
Der Hautarzt; Zeitschrift für Dermatologie, Venerologie, und verwandte Gebiete
Publication Date
Sep 01, 1997
Volume
48
Issue
9
Pages
653–656
Identifiers
PMID: 9410851
Source
Medline
License
Unknown

Abstract

The association of nevus flammeus with mongolian spot, nevus fuscoceruleus, nevus spilus and, with variable frequency, with nevus anemicus has been termed phacomatosis pigmentovascularis, a genodermatosis first described by Ota and co-workers. Four different combinations have been specified. Most cases are reported from the Japanese literature. Phacomatosis pigmentovascularis may constitute an exclusively cutaneous disorder, but overlapping with other syndromes like Klippel-Trenaunay syndrome or Sturge-Weber syndrome is also possible. We report a 30-year-old woman with nevus flammeus on the back and right arm associated with nevus fuscoceruleus on the back.

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