Erdheim-Chester Disease: A Rare Clinical Entity
  • Margarida Oliveira
    Department of Medicine, Hospital Pedro Hispano, Unidade Local de Saúde de Matosinhos, Matosinhos, Portugal
  • Sofia Monteiro
    Department of Medicine, Hospital Pedro Hispano, Unidade Local de Saúde de Matosinhos, Matosinhos, Portugal
  • Joana dos Santos
    Pathology Department, Hospital Pedro Hispano, Unidade Local de Saúde de Matosinhos, Matosinhos, Portugal
  • Ana Catarina Silva
    Radiology Department, Hospital Pedro Hispano, Unidade Local de Saúde de Matosinhos, Matosinhos, Portugal
  • Rute Morais Ferreira
    Department of Medicine, Hospital Pedro Hispano, Unidade Local de Saúde de Matosinhos, Matosinhos, Portugal

Keywords

Erdheim-Chester disease, pericardial effusion, retroperitoneal space

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.

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    Published: 2020-06-30
    Issue: 2020: Vol 7 No 9 (view)

    How to cite:
    1.
    Oliveira M, Monteiro S, dos Santos J, Silva AC, Morais Ferreira R. Erdheim-Chester Disease: A Rare Clinical Entity. EJCRIM 2020;7 doi:10.12890/2020_001630.