Gsell-Erdheim syndrome: main pathomorphological alterations
Zerbino, D.D.; Kuzyk, I.I.; Gavryliuk, E.M.
Arkhiv Patologii 65(6): 36-40
2003
ISSN/ISBN: 0004-1955 PMID: 14964967 Document Number: 562707
24 cases of Gsell-Erdheim syndrome observed in 1999-2001 have been analysed. There were 20 men (83.4%) and 4 women (16.6%) aged 22-69 years. Six men (25%) died at the age under 50 years. At necropsies the following characteristic variants of stratification were observed: a total damage with stratification of the aorta at all length or, less often, local damage. Destruction of an elastic skeleton of the media is the basis of aorta stratification: zones free of the nuclei, foci of elastolysis (medianecrosis), thinning, dyschromia and fragmentation of elastic fibers, focal hyperelastosis. The pathological process involves all the aortic length not only the area of aneurysm and rupture. Manifestations of variable severity of the same process were seen in different parts of the aortic wall.