EHLERS-DANLOS SYNDROME TYPE-IV - A HETEROGENEOUS DISEASE

The Ehlers-Danlos syndrome is an inherited disorder of connective tiss ue, consisting of al least 10 different clinical subtypes. Type IV Ehl ers-Danlos syndrome is an autosomal dominant condition characterized b y the joint and dermal manifestations as in other forms of the syndrom e but also by the proneness to spontaneous rupture of bowel and large arteries. The authors describe their experience with three patients pr esenting type IV Ehlers-Danlos syndrome: the first presented with seve ral subsequent arterial ruptures, the second with multiple aneurysms, and the third with a dissection of the internal carotid artery. Clinic al features, incidence, diagnosis, and treatment of the syndrome are d iscussed.