Erdheim-Chester disease: MR imaging, anatomic, and histopathologic correlation of orbital involvement AMERICAN JOURNAL OF NEURORADIOLOGY de Abreu, M. R., Chung, C. B., Biswal, S., Haghighi, P., Hesselink, J., Resnick, D. 2004; 25 (4): 627-630


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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View details for PubMedID 15090356