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American Journal of Neuroradiology 25:627-630, April 2004
© 2004 American Society of Neuroradiology

Case Report
HEAD AND NECK

Erdheim-Chester Disease: MR Imaging, Anatomic, and Histopathologic Correlation of Orbital Involvement

Marcelo R. de Abreua, Christine B. Chungb, Sandip Biswalb, Parvis Haghighib, John Hesselinkb and Donald Resnickb

a Department of Radiology, Hospital Mae de Deus e Mae de Deus Center, Porto Alegre, Brazil
b Department of Radiology, University of California, San Diego, and Veterans’ Administration Health Care System, La Jolla, CA

Address correspondence to Christine B. Chung, MD, Department of Radiology, University of California, San Diego, and Veterans’ Administration Health Care System, 3350 La Village Dr., La Jolla, CA 92161

Summary: Erdheim-Chester disease (ECD) is a rare form of histiocytosis of unknown origin characterized by tissue infiltration by lipid-laden histiocytes. Typically, the diaphyseal and metaphyseal portions of the tubular bones are affected, leading to a characteristic radiographic pattern of bone sclerosis. Orbital involvement is not infrequent and is manifested by exophthalmos and periorbital xanthomatous lesions, with associated visual problems. This case report documents imaging and pathologic findings in a patient with ECD with extensive orbital involvement.




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Mayo Clin. Proc., April 1, 2005; 80(4): 546 - 548.
[Abstract] [PDF]


Copyright © 2009 by the American Society of Neuroradiology. Print ISSN: 0195-6108 Online ISSN: 1936-959X