Figure 1

Findings in a patient with unknown bullous keratopathy (Case 1). A. Slit-lamp photograph showing diffused corneal edema. B and C. In vivo confocal microscopy of the endothelial layer showing abundant irregular hyperreflective materials most likely PEX materials in the endothelium (B, arrows; Bar = 50 μm) and stroma of the cornea (C, arrows; Bar = 50 μm). D and E. Hematoxylin-eosin staining of light microscopy showing apparent loss of endothelium and formation of fibrillar layer (D, double arrows) and the fibroblast-like change of an endothelium (E). St: stroma, De: Descemet membrane, Bar = 10 μm. F and G. Scanning electronic microscopy showing PEX fibers (open arrows) adjacent to destroyed endothelium (F; En: endothelium) and transmission electron microscopic identification of PEX fibers (arrows in enlarge window) in the thickened Descemet membrane (G; St: stroma, De: Descemet membrane, original magnification: x5000). H. Anti-LOXL1 immunohistochemical staining showing positive staining within the stroma of the cornea (arrows). St: stroma, De: Descemet membrane, Bar = 10 μm. Window image shows negative control staining without LOXL1 antibody.