Eosinophilic Granuloma (Histiocytosis X) Clinical Presentation

  • Author: Eleanor M Summerhill, MD, FACP, FCCP; Chief Editor: Zab Mosenifar, MD   more...
 
Updated: Jan 17, 2012
 

History

Presentations of pulmonary Langerhans cell histiocytosis X (PLCH) are variable. Approximately 25% of patients are asymptomatic, and their disease is diagnosed after an evaluation of incidental findings on chest radiographs. Others present with respiratory or constitutional symptoms. In order of decreasing frequency, common presenting symptoms are as follows:

  • Nonproductive cough (56-70%)
  • Dyspnea (40%)
  • Fatigue (30%)
  • Weight loss (20-30%)
  • Chest pain (21%)
  • Spontaneous pneumothorax, which may be recurrent, is a classic presentation found in 10-20% of patients.
  • Fever (15%)
  • Cystic bone lesions (4-20%): These may be painful and may predispose the patient to pathologic fracture.
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Physical

Patients with pulmonary Langerhans cell histiocytosis X (PLCH) present with nonspecific physical findings. Neither inspiratory rales (crackles) nor clubbing is common. Cor pulmonale may develop; therefore, the following related findings may be present:

  • Loud second heart sound with accentuated pulmonic component
  • Tricuspid regurgitation murmur
  • Right ventricular lift
  • Peripheral edema

Also see a clinical guideline summary from the American College of Chest Physicians, Uncommon causes of cough: ACCP evidence-based clinical practice guidelines.[8]

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Causes

No occupational causes or geographic predispositions are recognized for pulmonary Langerhans cell histiocytosis X (PLCH). People with PLCH, almost invariably, are cigarette smokers. Antigenic stimulation from 1 or more components of tobacco smoke is likely responsible for the disease. Because only a few tobacco smokers develop the disease, other susceptibility factors, such as host genetics and environmental exposures, most likely play an important role in pathogenesis. Some reports in the literature also describe PLCH developing following radiation and/or chemotherapy for lymphoma. Additional investigation is needed to further our understanding of this disease process.

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Contributor Information and Disclosures
Author

Eleanor M Summerhill, MD, FACP, FCCP  Associate Professor of Medicine, Division of Pulmonary and Critical Care Medicine, Warren Alpert Medical School of Brown University; Director, Internal Medicine Residency Program, Memorial Hospital of Rhode Island

Eleanor M Summerhill, MD, FACP, FCCP is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, American Thoracic Society, Association of Program Directors in Internal Medicine, and Rhode Island Medical Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Michael Peterson, MD  Chief of Medicine, Vice-Chair of Medicine, University of California, San Francisco, School of Medicine; Endowed Professor of Medicine, University of California, San Francisco-Fresno, School of Medicine

Michael Peterson, MD is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, and American Thoracic Society

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Daniel R Ouellette, MD, FCCP  Associate Professor of Medicine, Wayne State University School of Medicine; Consulting Staff, Pulmonary Disease and Critical Care Medicine Service, Henry Ford Health System

Daniel R Ouellette, MD, FCCP is a member of the following medical societies: American College of Chest Physicians and American Thoracic Society

Disclosure: Nothing to disclose.

Timothy D Rice, MD  Associate Professor, Departments of Internal Medicine and Pediatrics and Adolescent Medicine, St Louis University School of Medicine

Timothy D Rice, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Physicians

Disclosure: Nothing to disclose.

Chief Editor

Zab Mosenifar, MD  Director, Division of Pulmonary and Critical Care Medicine, Director, Women's Guild Pulmonary Disease Institute, Professor and Executive Vice Chair, Department of Medicine, Cedars Sinai Medical Center, University of California, Los Angeles, David Geffen School of Medicine

Zab Mosenifar, MD is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, American Federation for Medical Research, and American Thoracic Society

Disclosure: Nothing to disclose.

Additional Contributors

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author, Robert S. Crausman, MD, MMS, to the development and writing of this article.

References

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Low-power photomicrograph of a lung-tissue specimen that demonstrates the classic stellate nodule of pulmonary histiocytosis X (hematoxylin-eosin stain).
 
 
 
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