抄録
Erdheim-Chester disease is a rare systemic histiocytic disorder. The authors present a case of a 65-year-old woman suffering from chronic pericardial effusion of undetermined etiology for several years. She was referred to us with weakness of the right extremities and speech disturbance. Neuroradiological studies showed multiple dura- based lesions. Huge right extra-cerebral lesion was removed and histopathological examination revealed a histiocytic proliferation positive for CD68 and negative for S100 with scattered Touton type giant cells, consistent with Erdheim-Chester disease. Plain X-ray showed pathognomonic sclerotic change of the meta/diaphyses of the femur and tibia. Erdheim-Chester disease should be considered in a differential diagnosis of dura-based lesions.
