Cardiovascular manifestations of Pseudoxanthoma elasticum.

摘要: Autopsies were performed in three cases of pseudoxanthoma elasticum (PXE) to evaluate the cardiovascular changes. The endocardial lesion characterized by intimal fibroelastotic thickening and disorganization, fragmentation, calcification elastic fibers deeper layers is unique histologically. Severe atherosclerosis was present all resembled that encountered routinely. Fragmentation degeneration laminae muscular arteries followed vascular could not be distinguished morphologically from Monckeberg's arteriosclerosis. All showed striking initimal thickening, particularly intrarenal arteries, resembling seen hypertension, although only one subjects hypertensive. It would appear metabolic defect PXE predisposes premature onset accelerated development commonly aging processes, specific manifestation disease.