Acute Cardiac Tamponade in a 77-year-old Italian Woman with Erdheim-Chester Disease

  • Maria Kyriakopoulou Cardiology Department, Cliniques Universitaires Erasme, Bruxelles
  • Guy Decaux Cardiology Department, Cliniques Universitaires Erasme, Bruxelles
  • Mike El Mourad Cardiology Department, Cliniques Universitaires Erasme, Bruxelles
  • Ruben Casado Arroyo Cardiology Department, Cliniques Universitaires Erasme, Bruxelles

Keywords

Erdheim Chester disease, tamponade, BRAF, interferon alpha

Abstract

Erdheim-Chester disease (ECD) is a non-Langerhans’ histiocytosis and a very rare multisystemic disease of unknown aetiology, with skeletal involvement of the long bones and in more than 50% of cases with extraskeletal involvement. The disease was described in 1930 by the anatomopathologist Jakob Erdheim and his student William Chester. More than 500 cases have since been reported. We report the case of a 77-year-old Italian woman with ECD who was admitted to hospital for acute cardiac tamponade. The patient presented with simultaneous cutaneous, retro-orbital, skeletal, cerebral and cardiovascular manifestations and was successfully treated with corticosteroids followed by interferon.

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References

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  • Published: 2016-11-28

    Issue: Vol. 3 No. 7 (view)

    Section: Articles

    How to cite:
    1.
    Kyriakopoulou M, Decaux G, El Mourad M, Casado Arroyo R. Acute Cardiac Tamponade in a 77-year-old Italian Woman with Erdheim-Chester Disease. EJCRIM 2016;3 doi:10.12890/2016_000451.