Respiratory Medicine Case Reports (Jan 2023)

A recurrent pleuropneumonia revealing Erdheim-Chester Disease

  • Yousra Kherabi,
  • Thibaud Chazal,
  • Jean-François Emile,
  • Wassim Allaham,
  • Elise Mallart,
  • Victoire de Lastours,
  • Julien Haroche,
  • Yann Nguyen

Journal volume & issue
Vol. 43
p. 101843

Abstract

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Erdheim-Chester disease (ECD) is a rare form of L group histiocytosis, accounting for up to 1500 cases to date worldwide, which mainly affects men between their 5th and 7th decade of life. The most frequent manifestations are bone involvement, perirenal infiltration with an evocating appearance of “hairy kidneys”, and a “coated aorta” aspect. Lung involvement in ECD is less common and includes pleural infiltration and interstitial lung disease. Herein, we report the case of a 76-year-old woman with recurrent pleuropneumonia revealing ECD.

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