Pulmonary Langerhans cell histiocytosis

Case contributed by Liz Silverstone β—‰ β—ˆ


Prior pleurodesis for pneumothorax in ex-smoker.

Patient Data

Age: 50 years
Gender: Male
  • numerous irregular rounded cysts with upper zone peribronchial predominance and sparing the costophrenic and anterior angles

  • high-attenuation pleural thickening bilaterally due to VATS pleurodesis

  • no nodules

  • no bone lesions

Case Discussion

Typical history and features of inactive pulmonary Langerhans cell histiocytosis (PLCH), an inhalational lung disease. Bronchial wall destruction and bronchial dilatation is responsible for the appearance and location of the cysts. Nodules are a feature of active inflammation of the bronchial walls and are not present in this case.

Smoking cessation is the mainstay of disease control and serial follow-up with pulmonary function tests +/- CT may be helpful.

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