This 26 year old non-smoker woman presented with a spontaneous pneumothorax at 3 months ago. The pneumothorax was treated by simple aspiration resulting in adequate clinical and radiological improvement. However, 3 months later, she presented with further pneumothorax. She had no signs of tuberculosis, rheumatic disease and other background diseases. A lung HRCT scan showed extensive cystic air spaces throughout both lungs on suspicion of lymphangioleiomyomatosis (LAM) (Fig. 1). In view of her age a thoracoscopic lung biopsy was performed in July 2013. At operation there were numerous cystic areas within all lobes of the right lung and she had a large apical bulla. There were no post-operative complications and the lung remained fully expanded. Histology of the lung showed smooth muscle, staining for desmin, HMB-45, beta-catenin, SMA, ER, and PR, within the lung, alveolar walls and blood vessels not in keeping with LAM. However, there are diffuse pulmonary interstitial cholesterol clefts surrounded by lymphocytes, without the formation of granulomas (Fig. 2).
HRCT shows extensive cystic air spaces throughout both lungs with right pneumothorax.