Multimodality imaging showing complete cardiovascular involvement by Erdheim-Chester disease

Abstract

Erdheim-Chester disease (ECD) is a multisystem non-Langerhans form of cell histiocytosis. Histiocytic infiltration leads to xanthogranulomatous infiltrates of multiple organ systems. Erdheim-Chester disease was first reported in 1930, only 320 cases reported in the literature. Cardiac involvement in ECD carries worst prognosis beside the central nervous system. We report the first case with pan-cardiac involvement diagnosed with multimodality imaging.