[Abdominal emergencies in type IV ehlers-Danlos syndrome]

Gastroenterol Clin Biol. 1998 Mar;22(3):343-5.
[Article in French]


Ehlers-Danlos syndrome denotes a group of inherited connective tissue diseases comprising nine types. Type IV Ehlers-Danlos syndrome is the most life-threatening form. It is characterized by a type III collagen deficiency resulting in arterial fragility and death from vascular rupture or bowel perforation. This disease involves a col 3A1 gene mutation. We report the case of a 44 year-old woman with type IV Ehlers-Danlos syndrome. The medical history of our patient included bowel necrosis and two vascular ruptures. We indicate data required to establish Ehlers-Danlos syndrome diagnosis and guidelines for patient management.

Publication types

  • Case Reports
  • English Abstract

MeSH terms

  • Adult
  • Collagen / deficiency
  • Digestive System / pathology
  • Ehlers-Danlos Syndrome / complications*
  • Ehlers-Danlos Syndrome / pathology
  • Ehlers-Danlos Syndrome / surgery
  • Female
  • Gastrointestinal Hemorrhage / complications
  • Humans
  • Laparotomy


  • Collagen