Case of von Recklinghausen disease complicated with a solitary lung lesion located mainly in the upper lung field

A 72-year-old man presented with grade III dyspnea according to the Hugh-Jones scale in February, 2007, and he was referred to our department. Massive fibrosis of upper lung field dominance and bilateral pleural effusion were observed on chest X-ray films and CT. A respiratory function test revealed mixed ventilatory disturbance. Thereafter, left-sided pneumothorax developed in February 2008, followed by right-sided pneumothorax. He recovered once; however, respiratory failure progressed. He was re-hospitalized due to pneumonia and CO2 narcosis. Respiratory failure could not be prevented, and he died in September 2008. Macroscopic autopsy findings included fibrous adhesion of the pleura and fibrous consolidation of lung parenchyma which was most dominant in the bilateral apices. These were accompanied by bronchiectasis and brochiolectasis. Microscopically, the core pathology was organizing bronchiolitis and organizing pneumonia. Reported cases of diffuse lung lesions complicated with von Recklinghausen disease mostly comprise fibrosis and emphysematous changes. The unique pathological findings in this case seemed unrelated to any known disease entity.