Erdheim-Chester Disease: A Case of a Cardiac Involvement

Abstract

Erdheim-Chester disease (ECD) is a rare non-Langerhans histiocytic multisystem disorder due to xanthogranulomatous infiltration of tissues by spumous histiocytes, It typically presents with diffuse bone pain however the cardiac manifestation are common and occur to 75% of the patients. It is frequently asymptomatic and detected incidentally on radiological imaging and it most commonly manifests as pericardium and myocardium infiltration We report the case of a 66-year-old woman patient, admitted for diffuse bone pain, asthenia and a left arm weakness who presented and infiltration of the aorta with a coated aspect, a thickened right atrium and a small pericardial effusion.