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

Abstract

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.