Pulmonary Langerhans cell histiocytosis concurrent with bronchial hyperresponsiveness on a nonsmoker adult presenting with spontaneous pneumothorax

SummaryWe report a nonsmoker adult with histologically proven pulmonary Langerhans cell histiocytosis (PLCH) with bronchial hyperresponsiveness (BHR) presenting with spontaneous pneumothorax. An 18-year-old, nonsmoker student had shortness of breath for a decade. PFTs and lung volume study (obtained one month post-operation) showed a mild obstructive airway with bronchodilator response and a decreased DLco with a positive methacholine challenge test (PC20<8 mg/ml). Chest radiograph revealed right-sided pneumothorax and diffuse reticular infiltrates. HRCT revealed scattered, thin-walled lung cysts in all lobes without sparing of the costophrenic angles. The patient underwent a thoracotomy for wedge resection and the diagnosis of PLCH was confirmed by a demonstration of the accumulated Langerhans cells histiocytes (positive CD1a) in lung tissue. Pneumothorax was managed by bleblectomy and pleurodesis. This case report demonstrates BHR concurrent with PLCH on a nonsmoker patient. The combination of BHR and PLCH may confer a detrimental synergistic effect on the respiratory symptoms, deterioration of lung function, lung cysts and hence spontaneous pneumothorax on these patients.