Malignant atrophic papulosis (Q104321): Difference between revisions

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Latest revision as of 10:10, 17 August 2026

Degos' disease (malignant atrophic papulosis) is a rare, progressive occlusive vasculopathy of unknown cause affecting small and medium calibre arteries. Its protean manifestations result from focal tissue infarction in skin and other organs. It presents initially in the skin as scattered small pink papules which undergo ischaemic necrosis to leave characteristic circular porcelain-white scars. Although it may remain limited to the skin, it tends over time to progress to involve other organ systems. Multiple focal infarcts in the CNS may result in a wide variety of neurological sequelae. Gastrointestinal bleeding and perforation are the most common causes of death.

Language	Label	Description	Also known as
default for all languages	ID_792094526
English	Malignant atrophic papulosis	Degos' disease (malignant atrophic papulosis) is a rare, progressive occlusive vasculopathy of unknown cause affecting small and medium calibre arteries. Its protean manifestations result from focal tissue infarction in skin and other organs. It presents initially in the skin as scattered small pink papules which undergo ischaemic necrosis to leave characteristic circular porcelain-white scars. Although it may remain limited to the skin, it tends over time to progress to involve other organ systems. Multiple focal infarcts in the CNS may result in a wide variety of neurological sequelae. Gastrointestinal bleeding and perforation are the most common causes of death.