Preferred Label : Malignant atrophic papulosis;
ICD-11 definition : 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.;
ICD-11 synonym : maladie de degos; erythrokeratoderma en cocardes; kohlmeier-degos syndrome; Degos disease; malignant atrophic papulosis of degos; Lethal Cutaneous and Gastrointestinal Arteriolar Thrombosis;
ICD-11 inclusion : Degos disease;
Origin ID : 792094526;
Currated CISMeF NLP mapping
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.
