Pulmonary Manifestations in von Recklinghausen''s Disease

Von Recklinghausen's disease, neurofibromatosis type 1 (NF1), is a commonautosomal disorder characterized by multiple neurofibromas, cafe au lait spots, and Lisch nodules of the iris with a variable clinical expression. This disorder appears to affect all races, with a prevalence estimated to be 1 in 3,000 live births. The NF1 gene is located in 17ql 1.2. However, due to the high mutation rate at the NF1locus, most patients are expected to have different mutations (1-3). Lung involvement in NF1 is a well-known complication, and it consists of diffuse interstitial fibrosis and bullae, either alone or in combination. The interstitial fibrosis characteristi-