Title |
Erdheim-Chester disease: description of eight cases
|
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Published in |
Clinical Rheumatology, April 2016
|
DOI | 10.1007/s10067-016-3269-y |
Pubmed ID | |
Authors |
S. Roverano, J. Gallo, A. Ortiz, N. Migliore, Mónica Eletti, S. Paira |
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. |
X Demographics
Geographical breakdown
Country | Count | As % |
---|---|---|
United States | 10 | 20% |
India | 5 | 10% |
Spain | 3 | 6% |
Turkey | 2 | 4% |
Mexico | 2 | 4% |
France | 2 | 4% |
Trinidad and Tobago | 1 | 2% |
Peru | 1 | 2% |
Indonesia | 1 | 2% |
Other | 7 | 14% |
Unknown | 17 | 33% |
Demographic breakdown
Type | Count | As % |
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Members of the public | 31 | 61% |
Practitioners (doctors, other healthcare professionals) | 13 | 25% |
Scientists | 4 | 8% |
Science communicators (journalists, bloggers, editors) | 3 | 6% |
Mendeley readers
Geographical breakdown
Country | Count | As % |
---|---|---|
Unknown | 13 | 100% |
Demographic breakdown
Readers by professional status | Count | As % |
---|---|---|
Professor | 4 | 31% |
Other | 2 | 15% |
Student > Bachelor | 2 | 15% |
Student > Postgraduate | 2 | 15% |
Researcher | 2 | 15% |
Other | 1 | 8% |
Readers by discipline | Count | As % |
---|---|---|
Medicine and Dentistry | 11 | 85% |
Unknown | 2 | 15% |