A 22-year-old male basketball player with back pain is found to have a dissecting aortic aneurysm. the follow is/are true:

a. In Marfan’s syndrome, a disorder of type I collagen underlies the observed cystic medial necrosis

b. In type IV Ehlers-Danlos syndrome, little or no type III collagen is produced and arterial rupture is likely

d. In arteria magna syndrome, the media is devoid of elastic tissue and coronary artery disease is common

Cystic medial necrosis is associated with aortic dissection at an early age and can be due to Marfan’s syndrome with its disorder of type I collagen or type IV Ehlers-Danlos where little or no type III collagen is produced. In pseudoxanthoma elasticum, the medial elastic tissue is replaced by calcific deposits and there are xanthoma-like cutaneous papules. In arteria magna syndrome, elastic tissue is absent in the media and associated coronary artery disease is common.