Malignant Atrophic Papulosis
Synonyms
Atrophic Papuloses, Malignant
Atrophic Papulosis, Malignant
Degos Disease
Degos Syndrome
Degos's Malignant Atrophic Papulosis
Disease, Degos
Disease, Kohlmeier-Degos
Erythrokeratoderma en cocardes
Kohlmeier-Degos Disease
Malignant Atrophic Papuloses
Papuloses, Malignant Atrophic
Papulosis, Malignant Atrophic
Syndrome, Degos
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.