Open AccessErdheim-Chester Disease: A Rare Clinical Entity
Author(s) -
Margarida Oliveira,
Sofia Monteiro,
Joana Dos Santos,
Ana Catarina Silva,
Rute Ferreira
Publication year - 2020
Publication title -
european journal of case reports in internal medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.125
H-Index - 1
ISSN - 2284-2594
DOI - 10.12890/2020_001630
Subject(s) - medicine , pericardial effusion , erdheim–chester disease , differential diagnosis , radiology , cardiac tamponade , histiocyte , pericardium , biopsy , histiocytosis , disease , pathology , surgery
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.