Pericardial effusion as a crucial presentation of Erdheim-Chester disease in a hemodialysis patient: an overlooked diagnosis.

[erdheim-chester disease]

Erdheim-Chester disease (ECD ) is a rare non-Langerhans cell histiocytosis whose hallmark is tissue infiltration by CD 68 -positive , CD 1 a-negative and usually S-100 protein-positive foamy non-Langerhans histiocytes and mononuclear cells . Here , we report a hemodialysis (HD ) patient who presented with fever and pericardial effusion . We performed pericardiocentesis with pericardial biopsy and the histological findings indicated ECD . We administered intravenous methylprednisolone pulse therapy (250 mg /d ) followed by oral prednisolone (50 mg /d ). The patient 's fever gradually subsided and there was no recurrence of pericardial effusion . This is the first report of an HD patient with ECD . We suggest that ECD be considered in the differential diagnosis of new HD patients who present with pericardial effusion , especially when this did not improve following increased dose of HD .