Orphanet: Phakomatosis pigmentovascularis

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Phakomatosis pigmentovascularis

Disease definition

A rare skin disease characterized by the co-occurrence of a widespread vascular nevus (typically nevus flammeus) and a pigmentary nevus, potentially associated with a variety of other cutaneous nevi, and with or without extracutaneous (most commonly central nervous system, ocular, or musculoskeletal) involvement. Several subtypes are distinguished based on phenotypic characteristics.


Classification level: Disorder
  • Synonym(s): -
  • Prevalence: -
  • Inheritance: Not applicable 
  • Age of onset: Neonatal
  • ICD-10: Q85.8
  • OMIM: -
  • UMLS: C1274879
  • MeSH: -
  • GARD: 4312
  • MedDRA: -
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