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: LATEST ONLINE (view)

    Section: Articles

    How to cite:
    Oliveira, M., Monteiro, S., dos Santos, J., Silva, A. C., & Morais Ferreira, R. (2020). Erdheim-Chester Disease: A Rare Clinical Entity. European Journal of Case Reports in Internal Medicine, 2. https://doi.org/https://doi.org/10.12890/2020_001630