Malignant Atrophic Papulosis

**Definition:** Variously described as a vasculopathy, endovasculitis, or occlusive arteriopathy, this condition occurs in a benign cutaneous form and a lethal multiorgan systemic variant. It is characterized by a narrowing and occlusion of the lumen of small to medium-sized blood vessels, leading to ischemia and infarction in the involved organ systems. The etiology and pathophysiology are unknown.

**Tree numbers:** - C14.907.137.520 - C14.907.940.530 - C17.800.862.457

**Synonyms:** - Degos Disease - Degos Syndrome - Degos's Malignant Atrophic Papulosis - Kohlmeier-Degos Disease - Papulosis, Malignant Atrophic