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Papers of the Week


2020


Eur J Case Rep Intern Med


7


9

Erdheim-Chester Disease: A Rare Clinical Entity.

Authors

Oliveira M, Monteiro S, Dos Santos J, Silva A C, Morais Ferreira R
Eur J Case Rep Intern Med. 2020; 7(9):001630.
PMID: 32908822.

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.