Diffuse plane xanthomatosis (Q55050): Difference between revisions
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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. | |||
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 |
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| 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. |
