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Radionuclide bone imaging in Erdheim-Chester disease.

Erdheim-Chester disease is a rare sporadic systemic histiocytic disease of unknown etiology that routinely involves the diametaphyseal region of the long bones of the appendicular skeleton. In addition, there is frequent involvement of multiple internal organs producing protean manifestations. Although definitive diagnosis requires histopathologic confirmation, its appearance on bone scintigraphy is virtually pathognomonic of this entity. We report the case of a 53-year-old man with Erdheim-Chester disease who presented with lower extremity bone pain and was found to have the characteristic lesions of this condition on bone scintigraphy.