Interstitial pulmonary fibrosis and von Recklinghausen's disease. An ultrastructural and immunofluorescent study

The clinical and pathologic findings in a 28 year old woman with pulmonary fibrosis and von Recklinghausen's disease are presented. Ultrastructural examination of a lung biopsy specimen showed increased collagen in the alveolar wall, associated with hyperplasia of granular pneumocytes. Large numbers of intraalveolar cells, morphologically suggestive of macrophages, but having tight junctions similar to epithelial cells were also present. Direct immunofluorescent examination using goat anti IgG, IgM, IgA, complement, albumin, and fibrinogen failed to show specific fluorescence. Despite certain structural similarities to other familial and idiopathic forms of interstitial pneumonia, the pathogenesis remains poorly understood.