Degos’ disease, also known as “malignant atrophic papulosis” is a rare vasculopathy characterized by typical cutaneous lesions with an unknown etiology which was first described by Dego in 1942 (1), but another case, reported in 1941 by Köhlmeier, who interpreted it as thromboangiitis obliterans of the mesenteric vessels (2).
It is an occlusive arteriopathy involving small-caliber vessels. Specifically, it is a progressive, small- and medium-size arterial occluding disease, leading to tissue infarction and initially involving the skin. Degos disease occurs both in a limited benign, cutaneous form and in a potentially lethal multiorgan, systemic variant.
The disease has a male predominance (3:1), and sporadic cases of familial involvement have been recorded. (3-7). The involvement of the gastrointestinal tract and other organs has been noted in approximately 60% of reported cases (8).
There are fewer than 50 living patients presently known worldwide, and fewer than 200 reported in medical literature. However, many individuals may go undiagnosed due to rarity of the disease (9,10).
Most individuals develop symptoms between the ages of 20-50; however, cases outside of this age range have been reported as well, even as early as 8 months (1,6).
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Moulin G. Les formes benignes de la papulose atrophiante maligna de Degos. [Benign forms of Degos’ malignant atrophic papulosis] Ann Dermatol Venereol. 1988; 115: 1289–1290.
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