Mucoid vasculopathy: vascular lesions in an autopsy study

Mucoid vasculopathy is a unique, idiopathic, vascular entity observed in autopsy material at the Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, India. The vascular lesions are the most prominent manifestation of a pathologic condition characterized by a generalized mucopolysaccharidosis of connective tissues found, for example, within nerve bundles in the adventitia of blood vessels and in the interstitium of such organs as the heart, lung, and kidney. Tissue deposits are accompanied by a mastocytosis. The pathology of vascular lesions observed in 44% of 131 autopsies is described. In this group, mucoid vasculopathy occurred more commonly in young males of lower socioeconomic status. Both arteries and veins are affected, particularly muscular arteries. They present a uniform concentric thickening of the intima and media, causing an apparent reduction in luminal diameter. Significant histological features include (a) large quantities of glycosaminoglycans accompanied by smooth muscle hyperplasia in the intima and media and (b) degenerative changes with mineralization of the internal elastic lamina of arteries and of mucoid material in the media. Except for the mucoid arteriopathy described in popliteal vessels in cases of idiopathic peripheral gangrene from Central Africa, which mucoid vasculopathy resembles somewhat, the latter condition is not exactly comparable to any other known vascular or connective tissue disorder.