Recurrent and rapidly occurring pericardial tamponade in 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.