Spontaneous pneumothorax with pulmonary Langerhans cell histiocytosis (PLCH) in an adult heavy cigarette smoker—A case report

Abstract

Abstract Pulmonary Langerhans cell histiocytosis is a rare disease caused by the proliferation of CD1a‐positive histiocyte‐like cells infiltrating the lung's interstitial layer. Most cases affect young to middle‐aged persons, especially adult heavy cigarette smokers. A 49‐year‐old male heavy smoker (40 pack‐year), with non‐productive cough, dyspnoea and desaturation, presented with a right‐sided pneumothorax on chest x‐ray with total atelectasis. Chest computed tomography (CT) revealed bilateral multiple thick‐walled infiltrated cysts and multiple ground‐glass nodules throughout the entire lung. Surgery with minimal invasive thoracoscopic lung biopsy and pleurodesis was performed. Pathology showed histiocyte‐like cells aggregates in the pulmonary parenchyma. Immunohistochemical stain demonstrated CD1a(+), S100(+) and CD68(+). After 3 months of smoking cessation, clear improvement was evidenced with a chest CT showing bilateral multiple thin‐walled rounded cysts and multiple ground‐glass nodules that are smaller in size and decreased in numbers. Early minimal invasive thoracoscopic lung biopsy and pleurodesis can also be a choice if the development of secondary spontaneous pneumothorax occurs.

Keywords

• CD1a‐positive histiocyte‐like cells
• ground‐glass nodules
• heavy cigarette smoker
• high‐resolution computed tomography (HRCT)
• lung biopsy
• lung‐infiltrated parenchyma cysts