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malignant atrophic papulosis

 

Definition: 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.

Synonym(s): Degos disease, Degos syndrome

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© Copyright 2017 Wolters Kluwer. All Rights Reserved. Review Date: Sep 19, 2016.
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