Erdheim-Chester disease: description of eight cases. Issue 6 (June 2016)

• Record Type: Journal Article
• Authors: Roverano, S.; Gallo, J.; Ortiz, A.; Migliore, N.; Eletti, Mónica; Paira, S.
• Is Part Of: Clinical rheumatology. Volume 35:Issue 6(2016)
• Page Start: 1625
• Page End: 1629
• Publication Date: 2016-06
• Subjects: Erdheim-Chester -- Non-Langerhans histiocytosis -- Xanthomatous tissue infiltration; Rheumatology -- Periodicals; 616.723
• DOI: 10.1007/s10067-016-3269-y
• Languages: English
• ISSNs: 0770-3198

Abstract

Abstract 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.