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.
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 .
What tissue is most affected in Ehlers-Danlos syndromes?
What tissue is most affected in Ehlers - Danlos syndromes ?
connective tissue
connective tissue