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Clin Imaging. 2009 Mar-Apr;33(2):150-3. doi: 10.1016/j.clinimag.2008.09.009.

Erdheim-Chester disease: case report with unique postmortem magnetic resonance imaging, high-resolution radiography, and pathologic correlation.

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  • 1Department of Radiology, Hospital Mae de Deus, Pedro Chaves Barcelos 1127/401, Porto Alegre RS 90450-010, Brazil.


Erdheim-Chester disease is an infiltrative form of histiocytosis characterized by replacement of normal tissues by lipid-laden histiocytes. The disease typically infiltrates the medullary portion of the diaphysis and metaphysis of long bones, producing a characteristic radiological pattern dominated by bone sclerosis. It usually affects adults of 40 years of age with a clinical spectrum ranging from an asymptomatic focal bone lesion to multisystemic disease. This case report documents unique imaging and pathologic findings of Erdheim-Chester disease using close postmortem pathologic-imaging correlation.

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