Ehlers - danlos syndrome

(Edvard Ehlers, 1863 - 1937, Danish dermatologist; Henri Alexandre Danlos, 1844 - 1912. French dermatologist), several rare complex abnormalities characterized by hyperelasticity and fragility of the skin and hypermobility of the joints. Cardiovascular abnormalities vary from nonexistent to severe. Mitral valve prolapse is the most frequent abnormality. The severest lesions occur in Ehlers Danlos type IV with a high frequency of spontaneous rupture of large and medium size arteries especially the aortic arch branches and the abdominal aorta and its branches. Pregnancy is hazardous in women with this syndrome because of a high incidence of arterial rupture. The arterial abnormalities may be demonstrated by X-ray aortography or selective arteriography. Echocardiography is the optimal technique for evaluating mitral valve prolapse and regurgitation. MRI and contrast-enhanced MR angiography can be used for identification and monitoring of aortic and arterial abnormalities associated with these syndromes.

CBH