Malignant atrophic papulosis (Q104321): Difference between revisions
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A doença de Degos (papulose atrófica maligna) é uma vasculopatia oclusiva progressiva rara, de causa desconhecida, que afeta artérias de pequeno e médio calibre. Suas manifestações variáveis resultam de infarto tecidual focal na pele e em outros órgãos. Apresenta-se inicialmente na pele como pequenas pápulas róseas espalhadas que sofrem necrose isquêmica e deixam cicatrizes circulares características, de cor branco-porcelana. Embora possa permanecer limitada à pele, tende a progredir com o tempo para envolver outros sistemas orgânicos. Múltiplos infartos focais no SNC podem resultar em uma ampla variedade de sequelas neurológicas. Hemorragia e perfuração gastrintestinal são as causas mais comuns de morte. | |||
| description / en | description / en | ||
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. | |||
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 |
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| 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. |
