Erdheim-Chester disease: description of eight cases
Erdheim-Chester disease, although rare, has a wide range of manifestations. It is characterized by the xanthomatous infiltration of tissues by spumous histiocytes, surrounded by fibrosis. The symptoms can vary from bone pain, diabetes insipidus, exophthalmos, xanthelasmas, cardiovascular involvement...
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| Veröffentlicht in: | Clinical rheumatology 2016-06, Vol.35 (6), p.1625-1629 |
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| container_title | Clinical rheumatology |
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| creator | Roverano, S. Gallo, J. Ortiz, A. Migliore, N. Eletti, Mónica Paira, S. |
| description | Erdheim-Chester disease, although rare, has a wide range of manifestations. It is characterized by the xanthomatous infiltration of tissues by spumous histiocytes, surrounded by fibrosis. The symptoms can vary from bone pain, diabetes insipidus, exophthalmos, xanthelasmas, cardiovascular involvement, bilateral adrenal enlargement, renal impairment, testis infiltration, interstitial lung disease to retroperitoneal fibrosis with perirenal and/or ureteral obstruction. We present eight cases, four of them with only breast involvement and the others with bone, cardiovascular, central nervous system, and renal involvement. All showed infiltrates of histiocytes and fibrosis on microscopic evaluation and positive CD68 and negative CD1a on immunohistochemical stains. |
| doi_str_mv | 10.1007/s10067-016-3269-y |
| format | Article |
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| source | MEDLINE; SpringerLink Journals (MCLS) |
| subjects | Abdomen - diagnostic imaging Adult Argentina Brief Report Erdheim-Chester Disease - diagnostic imaging Erdheim-Chester Disease - pathology Female Fibrosis Humans Male Medicine Medicine & Public Health Middle Aged Rheumatology Tomography, X-Ray Computed |
| title | Erdheim-Chester disease: description of eight cases |
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