[MIM*602248] a cutaneovisceral syndrome characterized by pathognomonic umbilicated porcelain-white papules with elevated telangiectatic anular borders, followed by the development of intestinal ulcers that perforate, causing peritonitis; arterioles in the lesions are occluded by thrombosis without inflammatory cells, leading to infarction, progressive neurological disability, and death. SYN: Degos disease, Degos syndrome.
