Diffuse plane xanthomatosis (Q55050): Difference between revisions
From determinar.ia.br - Determine suas informações
Created a new Item |
Changed an Item |
||||||||||||||
| (6 intermediate revisions by the same user not shown) | |||||||||||||||
| description / pt-br | description / pt-br | ||||||||||||||
Doença normolipidêmica rara na qual múltiplas máculas e placas xantomatosas planas se desenvolvem na pele em associação com uma paraproteinemia monoclonal. A doença geralmente ocorre em adultos, mas casos raros em crianças foram relatados. Os xantomas tipicamente envolvem pálpebras, pescoço, tronco superior, nádegas e flexuras. Os lipídios séricos geralmente são normais. Quase todos os pacientes têm uma paraproteína monoclonal circulante que em cerca de metade dos casos está associada a um transtorno mieloproliferativo, especialmente mieloma múltiplo, leucemia granulocítica ou linfocítica. Os xantomas surgem como resultado da deposição perivascular de complexos lipoproteína-imunoglobulina. | |||||||||||||||
| description / en | description / en | ||||||||||||||
This is a rare normolipidaemic disease in which multiple flat xanthomatous macules and plaques develop in the skin in association with a monoclonal paraproteinaemia. The disease generally occurs in adults but rare cases in children have been reported. The xanthomata typically involve the eyelids, neck, upper trunk, buttocks and flexures. Serum lipids are usually normal. Nearly all patients have a circulating monoclonal paraprotein which in about half the cases is associated with a myeloproliferative disorder, especially multiple myeloma, granulocytic or lymphocytic leukaemia. The xanthomata arise as a result of perivascular deposition of lipoprotein–immunoglobulin complexes. | |||||||||||||||
| Property / Canonical URI | |||||||||||||||
| Property / Canonical URI: https://id.who.int/icd/entity/461002025 / rank | |||||||||||||||
Normal rank | |||||||||||||||
| Property / CURIE | |||||||||||||||
CID11:ID_461002025 | |||||||||||||||
| Property / CURIE: CID11:ID_461002025 / rank | |||||||||||||||
Normal rank | |||||||||||||||
| Property / Canary Token | |||||||||||||||
dki-india-ID_461002025 | |||||||||||||||
| Property / Canary Token: dki-india-ID_461002025 / rank | |||||||||||||||
Normal rank | |||||||||||||||
| Property / Verification Status | |||||||||||||||
Concluído | |||||||||||||||
| Property / Verification Status: Concluído / rank | |||||||||||||||
Normal rank | |||||||||||||||
| Property / Knowledge Architect | |||||||||||||||
| Property / Knowledge Architect: https://pauloleads.com.br/cases-publicos/ / rank | |||||||||||||||
Normal rank | |||||||||||||||
| Property / Collection date | |||||||||||||||
13 August 2026
| |||||||||||||||
| Property / Collection date: 13 August 2026 / rank | |||||||||||||||
Normal rank | |||||||||||||||
Latest revision as of 02:41, 14 August 2026
This is a rare normolipidaemic disease in which multiple flat xanthomatous macules and plaques develop in the skin in association with a monoclonal paraproteinaemia. The disease generally occurs in adults but rare cases in children have been reported. The xanthomata typically involve the eyelids, neck, upper trunk, buttocks and flexures. Serum lipids are usually normal. Nearly all patients have a circulating monoclonal paraprotein which in about half the cases is associated with a myeloproliferative disorder, especially multiple myeloma, granulocytic or lymphocytic leukaemia. The xanthomata arise as a result of perivascular deposition of lipoprotein–immunoglobulin complexes.
| Language | Label | Description | Also known as |
|---|---|---|---|
| default for all languages | ID_461002025 |
||
| English | Diffuse plane xanthomatosis |
This is a rare normolipidaemic disease in which multiple flat xanthomatous macules and plaques develop in the skin in association with a monoclonal paraproteinaemia. The disease generally occurs in adults but rare cases in children have been reported. The xanthomata typically involve the eyelids, neck, upper trunk, buttocks and flexures. Serum lipids are usually normal. Nearly all patients have a circulating monoclonal paraprotein which in about half the cases is associated with a myeloproliferative disorder, especially multiple myeloma, granulocytic or lymphocytic leukaemia. The xanthomata arise as a result of perivascular deposition of lipoprotein–immunoglobulin complexes. |
Statements
CID11:ID_461002025
0 references
dki-india-ID_461002025
0 references
Concluído
0 references
13 August 2026
0 references
