Erdheim-Chester's Disease of the Heart: A Diagnostic Conundrum and Collision with the Same Mass in the Orbit

Abstract

Erdheim-Chester's disease is a rare multisystem xanthogranulomatosis, afflicting the skeletal system with the occasional involvement of soft tissues. We delineate an unusual case of a cardiac variant of Erdheim-Chester's disease presenting with pericardial effusion and as a collision with a synchronous orbital manifestation. We describe our diagnostic pathway and propose a novel treatment option involving nonsteroidal anti-inflammatory drugs. The role of cyclo-oxygenase in the disease process and inhibition thereof by NSAIDs is hypothesized and discussed.

Radiopharmaceutical diagnosis of Erdheim–Chester's disease

“…Utilizing both conventional and invasive diagnostic tools, the diagnosis of ECD was presumed and confirmed histopathologically [3]. Magnetic resonance imaging (MRI) justified xantho-granulomatous involvement of the orbit and ocular adnexa.…”

“…We have delineated a case of xantho-granulomatous disease involving the ocular tissues with atypical myocardial involvement [3]. A novel therapeutic approach, involving non-steroidal anti-inflammatory drugs, and a full work-up using radioisotopes to aid the diagnosis have also been proposed [4].…”

An Isotope-Diagnostic Approach to Erdheim-Chester's Disease of the Heart

“…A novel therapeutic approach involving nonsteroidal antiinflammatory drugs has also been proposed. 3 Because ECD has not been identified previously with noninvasive tests, herein we describe combined, whole-body isotope-diagnostic evaluation of our patient with ECD.…”