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Rare disease
CASE REPORT
An unusual case of interstitial lung disease in a patient with cardiopulmonary syndrome as the initial presentation of Erdheim-Chester disease
  1. Domingo Franco-Palacios1,
  2. April McDonald2,
  3. R Neal Aguillard3,
  4. Allen Berry4
  1. 1 University of Tennessee Health Science Center College of Medicine, Memphis, Tennessee, USA
  2. 2 Pulmonary and Critical Care, University of Tennessee, Memphis, Tennessee, USA
  3. 3 Pulmonary and Critical Care, Methodist University Hospital, Memphis, Tennessee, USA
  4. 4 Department of Pathology, Saint Francis Hospital, Memphis, Tennessee, USA
  1. Correspondence to Dr Domingo Franco-Palacios, dfrancopalacios{at}gmail.com

Summary

Erdheim-Chester disease (ECD) is a very rare disorder with only approximately 600 cases reported in the literature. ECD has been recently reclassified as a histiocytic dendritic cell neoplasm. The clinical spectrum ranges from asymptomatic tissue accumulation of histiocytes to invasive tissue infiltration, which can cause fulminant multisystem failure. It typically presents with bone pain and constitutional symptoms. Extraosseous manifestations are not uncommon. ECD-associated interstitial lung disease has been described in 20%-35% of patients. Diagnosis is primarily by tissue biopsy and immunohistochemistry showing xanthogranulomas composed of foamy histiocytes that stain positive for CD68, CD14 and CD163 and negative for CD1á and langerin. We report a case of ECD in a young man with cardiopulmonary involvement who presented with haemoptysis and dyspnoea.

  • Radiology (diagnostics)
  • Cardiovascular System
  • Immunology
  • Interstitial Lung Disease

https://doi.org/10.1136/bcr-2017-220659

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    Footnotes

    • Contributors DF-P and AM collected the data, drafted and revised the manuscript. RNA is the primary pulmonologist and continues to follow the patient in the clinic. AB is the pathologist who diagnosed the condition. All authors reviewed the supporting reference and participated in the approval of the final version of the manuscript.

    • Competing interests None declared.

    • Patient consent Obtained.

    • Provenance and peer review Not commissioned; externally peer reviewed.