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Stoppacciaro, A., Ferrarini, M., Salmaggi, C., Colarossi, C., Praderio, L., Tresoldi, M., et al. (2006) Immunohistochemical evidence of a cytokine and chemokine network in three patients with Erdheim-Chester disease— Implications for pathogenesis. Arthritis and Rheumatism, 54, 4018-4022. doi:10.1002/art.22280

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• TITLE: Fever of unknown origin as the first manifestation of Erdheim-Chester disease

  AUTHORS: Ning Li, Mingquan Chen, Huaping Sun, Yun Bao, Jiming Zhang

  KEYWORDS: Erdheim-Chester Disease; Histiocytosis; Osteoarthropathy

  JOURNAL NAME: Case Reports in Clinical Medicine, Vol.2 No.6, August 28, 2013

  ABSTRACT: Erdheim-Chester disease (ECD) is an uncommon non-Langerhans cell histiocytosis that affects multiple body systems and can present clinically in a myriad of ways. We report the case of a 29-year-old man presented with fever and diffuse bone pain. Physical examination showed bilateral and symmetrical long bone pain, especially the knees. Radiographs showed multiple mixed bone lesions with sclerotic and lytic areas of the femora, tibiae, and fibula. Biopsy of the tibial area displayed foamy lipid-laden histiocytes, confirming the diagnosis of ECD.