Malignant Atrophic Papulosis (Degos’ Disease)

Synonyms

Atrophic papulosquamous dermatitis; Degos’ disease; Fatal cutaneous-intestinal syndrome; Köhlmeier–Degos disease; Thromboangiitis cutaneointestinalis disseminata

Definition

Malignant atrophic papulosis (MAP) is a rare and often lethal, vaso-occlusive disorder involving small and medium-caliber vessels. It is characterized by narrowing and occlusion of the lumen by intimal proliferation and thrombosis, which leads to ischemia and infarction of the involved organ systems (most commonly the skin, gastrointestinal tract, and central nervous system). It was initially described by Köhlmeier in 1941 as a form of thromboangiitis obliterans, and recognized as a distinct vascular injury syndrome by Degos in 1942, hence the name (Thompson and Rosenbaum 2008).

Two variants have been described (Scheinfeld 2011). A cutaneous or benign variant, that is limited to the skin, with a relatively benign course. A systemic or malignant variant, in which there is a multiorgan involvement, with...