Erdheim-Chester Disease: A Rare Clinical Entity

Oliveira, Margarida and Monteiro, Sofia and dos Santos, Joana and Silva, Ana Catarina and Morais Ferreira, Rute (2020) Erdheim-Chester Disease: A Rare Clinical Entity. European Journal of Case Reports in Internal Medicine, 7 (9). pp. 1-4. ISSN ISSN: 2284-2594

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Abstract

Pericardial effusion represents a diagnostic challenge. Erdheim-Chester disease (ECD), though a rare cause, should be considered in the differential diagnosis. An 88-year-old woman was admitted to the hospital due to retrosternal pain, dyspnoea and constitutional symptoms. Hypoxaemic respiratory failure and increased inflammatory markers were documented. A chest x-ray revealed an increased cardiothoracic ratio. An echocardiogram showed a moderate-volume pericardial effusion, without signs of cardiac tamponade. A thoraco-abdomino-pelvic CT scan found a bilateral perirenal soft tissue halo. Perirenal mass biopsy showed diffuse infiltration by foamy histiocytes (CD68+), without IgG4, compatible with ECD. The correlation of anamnesis, radiology and histology is crucial for the diagnosis of ECD.

Item Type: Article
Uncontrolled Keywords: Erdheim-Chester disease, pericardial effusion, retroperitoneal space
Subjects: 600 Tecnologia - Scienze applicate
600 Tecnologia - Scienze applicate > 610 Medicina e salute (Classificare qui la tecnologia dei servizi medici)
600 Tecnologia - Scienze applicate > 610 Medicina e salute (Classificare qui la tecnologia dei servizi medici) > 616 Malattie (classificare qui la Clinica medica, la medicina basata sull'evidenza, la Medicina interna, la Medicina sperimentale) > 616.8 Malattie del sistema nervoso e disturbi mentali (Classificare qui la Neuropsichiatria, la Neurologia)
Depositing User: Marina Spanti
Date Deposited: 15 Dec 2021 13:19
Last Modified: 15 Dec 2021 13:19
URI: http://eprints.bice.rm.cnr.it/id/eprint/21001

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