CRCM  Vol.2 No.6 , September 2013
Fever of unknown origin as the first manifestation of Erdheim-Chester disease
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.
Cite this paper: Li, N. , Chen, M. , Sun, H. , Bao, Y. and Zhang, J. (2013) Fever of unknown origin as the first manifestation of Erdheim-Chester disease. Case Reports in Clinical Medicine, 2, 351-357. doi: 10.4236/crcm.2013.26095.

[1]   Aouba, A., Georgin-Lavialle, S., Pagnoux, C., Silva, N.M., Renand, A., Galateau-Salle, F., et al. (2010) Rationale and efficacy of interleukin-1 targeting in Erdheim-Chester disease. Blood, 116, 4070-4076. doi:10.1182/blood-2010-04-279240

[2]   Haroche, J., Arnaud, L. and Amoura, Z. (2012) Erdheim-Chester disease. Current Opinion in Rheumatology, 24, 53-59. doi:10.1097/BOR.0b013e32834d861d

[3]   Veyssier Belot, C., Cacoub, P., Caparros Lefebvre, D., Wechsler, J., Brun, B., Remy, M., et al. (1996) Erdheim-Chester disease—Clinical and radiologic characteristics of 59 cases. Medicine, 75, 157-169. doi:10.1097/00005792-199605000-00005

[4]   Wimpissinger, T.F., Schernthaner, G., Feichtinger, H. and Stackl, W. (2005) Compression of kidneys in Erdheim-Chester disease of retroperitoneum: Open surgical approach. Urology, 65, E29-E31. doi:10.1016/j.urology.2004.10.051

[5]   Chester, W. (1930) The lipogranulomatosis. Virchows Archiv für Pathologische Anatomie und Physiologie und für Klinische Medizin, 279, 561-602. doi:10.1007/BF01942684

[6]   Kairouz, S., Hashash, J., Kabbara, W., McHayleh, W. and Tabbara, I.A. (2007) Dendritic cell neoplasms: An overview. American Journal of Hematology, 82, 924-928. doi:10.1002/ajh.20857

[7]   Zelger, B.W.H., Sidoroff, A., Orchard, G. and Cerio, R. (1996) Non-Langerhans cell histiocytoses—A new unifying concept. American Journal of Dermatopathology, 18, 490-504. doi:10.1097/00000372-199610000-00008

[8]   Dion, E., Graef, C., Miquel, A., Haroche, J., Wechsler, B., Amoura, Z., et al. (2006) Bone involvement in Erdheirn-Chester disease: Imaging findings including periostitis and partial epiphyseal involvement. Radiology, 238, 632-639. doi:10.1148/radiol.2382041525

[9]   Allen, C.E. and McClain, K.L. (2011) Erdheim-Chester: Beyond the lesion. Blood, 117, 2745-2746. doi:10.1182/blood-2011-01-330233

[10]   Wright, R.A., Hermann, R.C. and Parisi, J.E. (1999) Neurological manifestations of Erdheim-Chester disease. Journal of Neurology, Neurosurgery & Psychiatry, 66, 72-75. doi:10.1136/jnnp.66.1.72

[11]   Perras, B., Petersen, D., Lorch, H. and Fehm, H.L. (2002) Psychoneuroendocrine disturbances in a patient with a rare granulomatous disease. Experimental and Clinical Endocrinology & Diabetes, 110, 248-252. doi:10.1055/s-2002-33075

[12]   Naqi, R., Azeemuddin, M., Idrees, R. and Wasay, M. (2010) Meningioma-like lesions in Erdheim Chester disease. Acta Neurochirurgica, 152, 1619-1621. doi:10.1007/s00701-010-0655-0

[13]   Donaldson, G., Bullock, P. and Monson, J.P. (2010) Erdheim-Chester disease mimicking multiple meningiomas. British Journal of Neurosurgery, 24, 296-297. doi:10.3109/02688691003624612

[14]   Arnaud, L., Pierre, I., Beigelman-Aubry, C., Capron, F., Brun, A.L., Rigolet, A., et al. (2010) Pulmonary involvement in Erdheim-Chester disease: A single-center study of thirty-four patients and a review of the literature. Arthritis & Rheumatism, 62, 3504-3512. doi:10.1002/art.27672

[15]   Haroche, J., Amoura, Z., Touraine, P., Seilhean, D., Graef, C., Birmele, B., et al. (2007) Bilateral adrenal infiltration in Erdheim-Chester disease. Report of seven cases and literature review. Journal of Clinical Endocrinology & Metabolism, 92, 2007-2012. doi:10.1210/jc.2006-2018

[16]   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

[17]   Dagna, L., Girlanda, S., Langheim, S., et al. (2010) Erdheim-Chester disease: Report on a case and new insights on its immunopathogenesis. Rheumatology, 49, 1203-1206. doi:10.1093/rheumatology/kep461

[18]   Braiteh, F., Boxrud, C., Esmaeli, B. and Kurzrock, R. (2005) Successful treatment of Erdheim-Chester disease, a non-Langerhans-cell histiocytosis, with interferon-alpha. Blood, 106, 2992-2994. doi:10.1182/blood-2005-06-2238

[19]   Jeon, I.-S., Lee, S.S. and Lee, M.K. (2010) Chemotherapy and Interferon-alpha treatment of Erdheim-Chester disease. Pediatric Blood & Cancer, 55, 745-747. doi:10.1002/pbc.22636

[20]   Suzuki, H.I., Hosoya, N., Miyagawa, K., Ota, S., Nakashima, H., Makita, N., et al. (2010) Erdheim-Chester disease: Multisystem involvement and management with interferon-alpha. Leukemia Research, 34, E21-E24. doi:10.1016/j.leukres.2009.07.026

[21]   Arnaud, L., Gorochov, G., Charlotte, F., Lvovschi, V., Parizot, C., Larsen, M., et al. (2011) Systemic perturbation of cytokine and chemokine networks in Erdheim-Chester disease: A single-center series of 37 patients. Blood, 117, 2783-2790. doi:10.1182/blood-2010-10-313510