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Capillary malformation-arteriovenous malformation

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Disease definition

This syndrome is characterised by the association of multiple capillary malformations (CM) with an arteriovenous malformation (AVM) and arteriovenous fistulas.

ORPHA:137667

Classification level: Group of disorders

Synonym(s):
  • CM-AVM

Source: PubMed ID 15917201

Prevalence: Unknown

Inheritance: Autosomal dominant, Not applicable

Age of onset: Infancy, Neonatal

ICD-10: Q27.3

ICD-11: LA90.3Y

OMIM: 608354 618196

UMLS: C1842180

MeSH: C564254

GARD: 11904

Summary
Epidemiology

So far, it has been described in multiple members of six families.

Clinical description

The CMs are atypical: they are small, round-to-oval in shape and pink-red in colour. AVMs may be cutaneous, subcutaneous, intramuscular, intraosseous or cerebral. The association of CM with arteriovenous fistulas or Parkes-Weber syndrome was reported in some cases.

Etiology

The syndrome is caused by heterozygous mutations in the RASA1 gene (5q13.3), encoding RAS p21 protein activator 1.

Last update: April 2008
A summary on this disease is available in Français, Español, Deutsch, Italiano, Português, Nederlands
Detailed information

Logo ERN: produced/endorsed by ERN(s) Logo FSMR: produced/endorsed by FSMR(s)

Guidelines
Emergency guidelines
Français (2018.pdf) - Orphanet Urgences
Clinical practice guidelines
English (2025) - J Vasc Anom (Phila) Logo ERN
English (2025) - Orphanet J Rare Dis
Disease review articles
Clinical genetics review
English (2019) - GeneReviews
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