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.
Also Known As:
Degos Syndrome; Degos's Malignant Atrophic Papulosis; Erythrokeratoderma en cocardes; Papulosis, Malignant Atrophic; Atrophic Papuloses, Malignant; Atrophic Papulosis, Malignant; Disease, Degos; Disease, Kohlmeier-Degos; Malignant Atrophic Papuloses; Papuloses, Malignant Atrophic; Syndrome, Degos; Degos Disease; Kohlmeier-Degos Disease