title: [Combined naevus flammeus and naevus fuscocoeruleus: phacomatosis pigmentovascularis type IIa].
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.
authors.affiliation: Klinik für Dermatologie und Venerologie, Medizinische Universität zu Lübeck.
authors.forename: W
authors.initials: W
authors.lastname: Achtelik
authors.forename: M
authors.initials: M
authors.lastname: Tronnier
authors.forename: H H
authors.initials: HH
authors.lastname: Wolff
mesh_terms: D000328:Adult; D001706:Biopsy; D003937:Diagnosis, Differential; D004416:Dysplastic Nevus Syndrome / Q000175:diagnosis / Q000235:genetics* / Q000473:pathology; D005260:Female; D006223:Hamartoma Syndrome, Multiple / Q000175:diagnosis / Q000235:genetics* / Q000473:pathology; D006801:Humans; D008544:Melanocytes / Q000473:pathology; D008854:Microscopy, Electron; D009507:Nevus of Ota / Q000235:genetics*; D010586:Phagocytes / Q000473:pathology; D012867:Skin / Q000473:pathology; D012878:Skin Neoplasms / Q000175:diagnosis / Q000235:genetics* / Q000473:pathology