Bronchiolitis obliterans with pemphigus vulgaris and castleman's disease of hyaline-vascular type: An autopsy case analyzed by computer-aided 3-D reconstruction of the airway lesions

Abstract We have experienced an autopsy case of a 20-year-old woman with bronchiolitis obliterans, pemphigus vulgaris, and Castleman's disease. The affected bronchioles showed almost the same histology as previously described. But, the number of the lesions seemed too small to cause her death of respiratory insufficiency. We also wondered whether or not some normal routes to the peripheral pulmonary tissues remained. After reviewing many histological sections from both lungs, one tissue block was chosen to make semiserial sections, and computer-aided three-dimensional (3-D) reconstruction analysis was performed. The 3-D reconstruction clearly showed two points: (1) Obstructive lesions developed within very narrow segments of bronchioles (0.4 to 0.7 mm in diameter; restricted to two to three generations) sparing terminal and respiratory bronchioles. This could explain the paucity of bronchiolar lesions that appeared on random sections; (2) All the affected bronchioles showed a high degree (up to 80–90%) of luminal stenosis. No intact routes to the periphery were left. Thus, respiratory insufficiency in this case became quite understandable.