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Familial multiple nevi flammei

Disease definition

Familial multiple nevi flammei is a rare, genetic capillary malformation disorder characterized by dark red to purple birthmarks which manifest as flat, sharply circumscribed cutaneous lesions, typically situated in the head and neck region, in various members of a single family. The lesions grow proportionally with the individual, change in color and often thicken with age.


Classification level: Disorder
  • Synonym(s):
    • Familial multiple port-wine stains
  • Prevalence: -
  • Inheritance: -
  • Age of onset: Neonatal
  • ICD-10: Q82.5
  • ICD-11: LC50
  • OMIM: 163000
  • UMLS: C2931029
  • MeSH: C535816
  • GARD: 3986
  • MedDRA: 10067193
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