ABDOMINAL EMERGENCIES OF TYPE-IV EHLERS-D ANLOS-SYNDROME

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 arteriel fragility and death form vascular ru pture or bowel perforation. This disease involves a col 3A1 gene mutat ion. We report the case of a 44 year-old woman with IV Ehlers-Danlos s yndrome. The medical history of our patient included bowel necrosis an d two vascular ruptures. We indicate data required to establish Ehlers -Danlos syndrome diagnosis and guidelines for patient management.