Erdheim-Chester disease (Q67672): Difference between revisions
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A doença de Erdheim-Chester é uma histiocitose Classe IIa (células não Langerhans) de etiologia desconhecida, caracterizada por uma infiltração xantogranulomatosa de vísceras, ossos, retroperitônio e pele por histiócitos carregados de lipídios e fibrose circundante. Apresenta-se mais comumente na vida adulta, com dor nos ossos, tipicamente ao redor dos joelhos e tornozelos. Exoftalmia, diabetes insipidus, xantoma cutâneo, fibrose pulmonar e uma ampla gama de complicações decorrentes da infiltração retroperitoneal e do SNC são todos bem reconhecidos. | |||
| description / en | description / en | ||
Erdheim-Chester disease is a Class IIa (non-Langerhans cell) histiocytosis of unknown aetiology characterised by a xanthogranulomatous infiltration of viscera, bones, retroperitoneum and skin by lipid-laden histiocytes and surrounding fibrosis. It most commonly presents in adult life with bone pain, typically around the knees and ankles. Exophthalmos, diabetes insipidus, cutaneous xanthomata, pulmonary fibrosis and a broad range of complications resulting from retroperitoneal and CNS infiltration are all well recognised. | |||
Revision as of 15:30, 14 August 2026
Erdheim-Chester disease is a Class IIa (non-Langerhans cell) histiocytosis of unknown aetiology characterised by a xanthogranulomatous infiltration of viscera, bones, retroperitoneum and skin by lipid-laden histiocytes and surrounding fibrosis. It most commonly presents in adult life with bone pain, typically around the knees and ankles. Exophthalmos, diabetes insipidus, cutaneous xanthomata, pulmonary fibrosis and a broad range of complications resulting from retroperitoneal and CNS infiltration are all well recognised.
| Language | Label | Description | Also known as |
|---|---|---|---|
| default for all languages | ID_1395439137 |
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| English | Erdheim-Chester disease |
Erdheim-Chester disease is a Class IIa (non-Langerhans cell) histiocytosis of unknown aetiology characterised by a xanthogranulomatous infiltration of viscera, bones, retroperitoneum and skin by lipid-laden histiocytes and surrounding fibrosis. It most commonly presents in adult life with bone pain, typically around the knees and ankles. Exophthalmos, diabetes insipidus, cutaneous xanthomata, pulmonary fibrosis and a broad range of complications resulting from retroperitoneal and CNS infiltration are all well recognised. |
