[Two cases of von Recklinghausen's disease with diffuse pulmonary changes]

Two patients with von Recklinghausen's disease had diffuse pulmonary diseases. The pulmonary diseases were interstitial fibrosis and emphysematous bullae. In case 2, examination of a specimen obtained by transbronchial lung biopsy revealed fibrotic interstitial thickening and adenomatous hyperplasia. Diffuse pulmonary abnormalities in von Recklinghausen's disease have been said to be fibrotic changes and emphysematous changes, as shown in roentgenograms in these two cases. The biopsy specimen obtained in case 2, however, revealed fibrotic changes only, which could not be distinguished from findings in cases of idiopathic interstitial pneumonia. In case 2, bronchoalveolar lavage fluid was also examined but it did not reveal any unique features of von Recklinghausen's disease. Since 1978, von Recklinghausen's disease has been diagnosed 10 times in our hospital, but diffuse pulmonary diseases was seen in only two of those 10 cases.