Erdheim disease

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Erdheim disease --> cystic medial necrosis

Loss of elastic and muscle fibres in the aortic media, with accumulation of mucopolysaccharide, sometimes in cystlike spaces between the fibres; a disease of unknown cause, which may be inherited and which predisposes to dissecting aneurysms.

Synonym: erdheim disease, medionecrosis aortae idiopathica cystica, medionecrosis of the aorta, mucoid medial degeneration.