Background: The report describes the case of a 20-year-old Saudi male patient with Pulmonary Langerhans cell histiocytosis X (PLCH). He presented with bilateral chest pain of few hours duration starting first on the right side. The patient had also dyspnea and non productive cough. The patient was heavy smoker for 6 years. General examination revealed that the patient had tachypnea but he was hemodynamically stable. Chest examination showed bilateral distant breath sounds but no hyperresonance. Chest x-rays revealed bilateral pneumothorax and high resolution CT scan of the chest revealed multiple bilateral pulmonary cysts. Oxygen is given to the patient and bilateral chest tubes were applied. Video assisted thoracic surgery was performed in 2 sessions with one week interval to resect the dominant blebs and to perform pleurodesis. The final histopathological diagnosis was pulmonary Langerhans cell histiocytosis X. Follow-up for more than 5 years revealed no recurrence. Pulmonary Langerhans cell histiocytosis X is a rare disease and may present by spontaneous pneumothorax, so, high index of suspicion is required in heavy smoker young males to diagnose the condition and to perform pleurodesis preventing recurrence of the pneumothorax which is expected to be high in PLCH.
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