Acute Cardiac Tamponade in a 77-year-old Italian Woman with Erdheim-Chester Disease.

Abstract

Erdheim-Chester disease (ECD) is a non-Langerhans’ histiocytosisand a veryrare multisystemic disease of unknown aetiology, with skeletal involvement of the long bones and in more than 50% of cases with extraskeletal involvement. The disease was described in 1930 by the anatomopathologist Jakob Erdheim and his student William Chester. More than 500 cases have since been reported. We report the case of a 77-year-old Italian woman with ECD who was admitted to hospital for acute cardiac tamponade. The patient presented with simultaneous cutaneous, retro-orbital, skeletal, cerebral and cardiovascular manifestations and was successfully treated with corticosteroids followed by interferon.LEARNING POINTSErdheim-Chester disease (ECD) is a non-Langerhans’ histiocytosisand a very rare multisystemic disease that is thought to be associated with cytokine disturbances.ECD has a variable prognosis but is poorer in those with heart involvement.First-line treatment involves the administration of interferon alpha.