Orphanet: Ehlers Danlos syndrome type 4

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Vascular Ehlers-Danlos syndrome

Disease definition

A rare genetic connective tissue disorder typically characterized by the association of unexpected organ fragility (arterial/bowel/gravid uterine rupture) with inconstant physical features as thin, translucent skin, easy bruising and acrogeric traits.


Classification level: Disorder
  • Synonym(s):
    • Arterial-ecchymotic EDS
    • EDS IV
    • Ehlers-Danlos syndrome type 4
    • Sack-Barabas syndrome
    • vEDS
  • Prevalence: Unknown
  • Inheritance: Autosomal recessive or Autosomal dominant 
  • Age of onset: Infancy, Neonatal
  • ICD-10: Q79.6
  • OMIM: 130050
  • UMLS: C0268338
  • MeSH: -
  • GARD: 2082
  • MedDRA: -
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