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Start free with EleplanFamilial multiple nevi flammei
ORPHA:624Morphological anomaly
Also called Familial multiple port-wine stains
What it is
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.
Key facts
- Age of onset
- Neonatal
- Inheritance
- Autosomal dominant
- Classified as
- Morphological anomaly
Signs and symptoms
Very common80–99%
4Sometimes5–29%
18- Abnormal cranial nerve morphology
- Abnormality of the lower limb
- Abnormality of the upper limb
- Arrhythmia
- Cerebral calcification
- Edema
- Glaucoma
- Hemiparesis
and 10 more in this range
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
Gene
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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