A histochemical study of a lung biopsy specimen from one patient revealed a marked increase of Gb3 that accounted for 10.7% of total lipids, and histopathologic examination revealed laminated inclusions in the capillary endothelium and type II pneumocytes [15]. Smith et al. [16] reported the histopathology and electron microscopy autopsy findings of the pulmonary vasculature in a 52 year-old man with Fabry disease. Zebra bodies inclusions were found within the endothelial cells of capillaries, arterioles and veins and in the smooth muscle cells of small pulmonary arteries. Zebra bodies with much thinner, delicate dense bands were also found in the lung parenchyma, either in the alveolar interstitium or within capillary endothelial cells.