A Case of Incidentally-diagnosed Erdheim-Chester Disease

Erdheim-Chester disease (ECD) is a rare multisystemic non-Langerhans cell histiocytosis that may be clonal and inflammatory in origin. The hallmark of the disease is infiltration of various organ systems by CD68+/CD1a- histiocytes containing foamy lipid-laden inclusions. The manifestations and course of the disease are variable and depend on the organ systems that are affected. Patients may be asymptomatic or may develop life-threatening complications, including myocardial infarction. The most common clinical manifestation is lower extremity bone pain. Imaging manifestations of the disease include symmetric osteosclerosis of the distal long bones, circumferentially “coated” aorta, pleural and pericardial thickening/fluid, and perirenal encasement. Treatment for the disease is evolving, particularly with the use of molecular BRAF inhibition. We present a case of a patient with ECD initially suspected based on the imaging manifestations.