Eosinophilic Granuloma (Histiocytosis X) Clinical Presentation
- Author: Eleanor M Summerhill, MD, FACP, FCCP; Chief Editor: Zab Mosenifar, MD, FACP, FCCP more...
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.
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
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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