Cystic medial necrosis



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

Cystic medionecrosis occurs in most cases independently, but often also within congenital connective tissue diseases, such as Marfan´s syndrome, Ehlers-Danlos syndrome or anuloaortic ectasia. The cause of this disease is unknown. Autosomal dominant inheritance is assumed.

Despite their name, the lesions described in cystic medial necrosis are not always necrotic a netvoří vždy cysty.

Cystical medial necrosis increases the risk of forming an aneurysm or anaortic dissection.

Related articles

 * Mucus dystrophy
 * Disorders of intercellular mass
 * Mukopolysacharidosis
 * Hereditary disorders of carbohydrate metabolism

Source

 * ws:https://www.wikiskripta.eu/index.php?curid=68571