Combined naevus flammeus and naevus fuscocoeruleus: phacomatosis pigmentovascularis type IIa

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 ca...

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Bibliographic Details
Published in:Hautarzt 1997-09, Vol.48 (9), p.653-656
Main Authors: Achtelik, W, Tronnier, M, Wolff, H H
Format: Article
Language:ger
Subjects:
Adult
Biopsy
Diagnosis, Differential
Dysplastic Nevus Syndrome - diagnosis
Dysplastic Nevus Syndrome - genetics
Dysplastic Nevus Syndrome - pathology
Female
Hamartoma Syndrome, Multiple - diagnosis
Hamartoma Syndrome, Multiple - genetics
Hamartoma Syndrome, Multiple - pathology
Humans
Melanocytes - pathology
Microscopy, Electron
Nevus of Ota - genetics
Phagocytes - pathology
Skin - pathology
Skin Neoplasms - diagnosis
Skin Neoplasms - genetics
Skin Neoplasms - pathology
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Description
Summary: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.
ISSN:0017-8470