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Clinical Rheumatology

, Volume 35, Issue 6, pp 1625–1629 | Cite as

Erdheim-Chester disease: description of eight cases

  • S. Roverano
  • J. Gallo
  • A. Ortiz
  • N. Migliore
  • Mónica Eletti
  • S. Paira
Brief Report

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.

Keywords

Erdheim-Chester Non-Langerhans histiocytosis Xanthomatous tissue infiltration 

Notes

Compliance with ethical standards

Disclosures

None.

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Copyright information

© International League of Associations for Rheumatology (ILAR) 2016

Authors and Affiliations

  • S. Roverano
    • 1
  • J. Gallo
    • 1
  • A. Ortiz
    • 1
  • N. Migliore
    • 2
  • Mónica Eletti
    • 2
  • S. Paira
    • 1
  1. 1.Rheumatology SectionJosé María Cullen HospitalSanta FeArgentina
  2. 2.Department of PathologyJosé María Cullen HospitalSanta FeArgentina

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