Erdheim-Chester's Disease of the Heart: A Diagnostic Conundrum and Collision with the Same Mass in the Orbit
DOI:
https://doi.org/10.1532/HSF98.20051109Abstract
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.References
Chester W. 1930. Über lipoidgranulomatose. Virchows Arch Pathol Anat 279:561-602.nCline MJ. 1994. Histiocytes and histiocytosis. Blood 84:2840-53.nDevouassoux G, Lantuejoul S, Chatelain P, Brambilla E, Brambilla C. 1998. Erdheim-Chester disease: a primary macrophage cell disorder. Am J Respir Crit Care Med 157:650-3.nKenn W, Stäbler A, Zachoval R, Zietz C, Raum W, Wittenberg G. 1999. Erdheim-Chester disease: a case report and a literature overview. Eur Radiol 9:153-8.nPetrikowski CG, McGaw WT. 2000. Erdheim-Chester disease of the jaws: literature review and case report. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 90:389-98.nSerratrice J, Granel B, De Roux C, et al. "Coated aorta": a new sign of Erdheim-Chester disease. J Rheumatol 27:1550-3.nHaroche J, Amoura Z, Dion E, et al. 2004. Cardiovascular involvement, an overlooked feature of Erdheim-Chester disease: report of 6 new cases and a literature review. Medicine (Baltimore) 83:371-92.nVeyssier-Belot C, Cacoub P, Caparros-Lefebvre D, et al. 1996. Erdheim-Chester disease: clinical and radiologic characteristics of 59 cases. Medicine (Baltimore) 75:157-69.nShiff SJ, Rigas B. 1999. The role of cyclooxygenase inhibition in the anti-neoplastic effects of nonsteroidal anti-inflammatory drugs (NSAIDs). J Exp Med 190:445-50.n