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Familial multiple nevi flammei

MedGen UID:
Concept ID:
Disease or Syndrome
Synonyms: CAPILLARY MALFORMATIONS, CONGENITAL, 1, SOMATIC, MOSAIC; Nevi Flammei; PORT-WINE STAIN; Port-wine stain familial multiple
SNOMED CT: Familial multiple port-wine stains (763714006); Familial multiple nevi flammei (763714006)
Gene (location): GNAQ (9q21.2)
Monarch Initiative: MONDO:0008094
OMIM®: 163000
Orphanet: ORPHA624


A rare genetic capillary malformation 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. There is evidence that congenital capillary malformations can be caused by somatic mosaic mutation in the GNAQ gene on chromosome 9q21. [from SNOMEDCT_US]

Clinical features

From HPO
Nevus flammeus
MedGen UID:
Concept ID:
Congenital Abnormality
A congenital vascular malformation consisting of superficial and deep dilated capillaries in the skin which produce a reddish to purplish discolouration of the skin.

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