Pulmonary Langerhans' Cell Histocytosis Presenting With Recurrent Pneumothorax in a Young Patient

Abstract

Pulmonary Langerhans' cell histiocytosis (PLCH) is a rare cystic interstitial lung disease that primarily affects young adults. It is caused by a disorder of myeloid dendritic cells [1]. We present a unique case of recurrent pneumothorax secondary to PLCH in a teenager. CASE PRESENTATION: A 16-year-old male patient was admitted to the hospital with a 2-week history of recurrent non-specific right sided chest pain, difficulty breathing and a dry cough. He smoked 8-10 cigarettes daily for the last two years. Systemic review and past history were unremarkable. On examination he was found to have decreased air entry bilaterally, with a hyper-resonant percussion note on the right side. Chest X ray and high resolution computerized tomography showed bilateral cystic changes with relative sparing of the lower zones, and a right sided pneumothorax with subcutaneous emphysema (figure 1, 2). This was initially managed with a right sided intercostal chest drain, resulting in expansion of his right lung. However, a few days later he had a recurrent ipsilateral pneumothorax. Unfortunately, he had a persistent air leak after insertion of a new chest drain. He therefore underwent video-assisted thoracoscopic surgery (VATS) with bullectomy, pleurectomy and a wedge lung biopsy.The histopathological review of the lung tissue showed areas of scaring with numerous Langerhans cells (positive for S-100, Langerin and CD1A) confirming PLCH with emphysematous cysts (figure 3). He had an FDG PET and bone scans which did not reveal any extrapulmonary involvement of LCH .