Recurrent and rapidly occurring pericardial tamponade in Erdheim Chester disease.

[erdheim-chester disease]

Erdheim Chester disease is a very rare histiocytic disorder characterised by tissue infiltration by lipid laden histiocytes . The most common presentation is bone pains typically involving the long bones . Over time almost 50 % of the patients develop extraosseous involvement . The prognosis depends on the extent and distribution of the extraskeletal manifestations . Cardiovascular involvement is seen in up to 40 % of the patients and the most common manifestations are periaortic fibrosis and pericardial involvement . Respiratory distress , extensive pulmonary fibrosis , and cardiac failure are the most common causes of death in these patients . Cardiac tamponade has also been documented to cause death in these patients . We describe a patient of Erdheim Chester disease who presented with recurrent and very rapidly occurring cardiac tamponade in a short duration of time and benefited from timely recognition and management .