Cystic medionecrosis

Cystic medianecrosis (also called Erdheim's disease, cystic medial necrosis or cystic medial degeneration) is a disease affecting the large arteries, especially the aorta, which is characterized by accumulation of mucopolysaccharides in the media of these vessels and loss of muscle and elastic fibers. These lesions resemble cysts in appearance.

In most cases, cystic medionecrosis occurs independently, but not infrequently also within congenital diseases of the connective tissue, e.g. Marfan's syndrome, Ehlers-Danlos syndrome or annuloaortic ectasia. The cause of this disease is unknown. An autosomal dominant inheritance is assumed.

Despite its name, the lesions described in cystic medianecrosis are not always necrotic and do not always form cysts.

Cystic medianecrosis increases the risk of developing aneurysm or aortic dissection.

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