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case_images: Images of the proband's right eye (patient II-1). (A-C): Corneal slit lamp biomicroscopy direct and indirect illumination by dilated pupil displays diffuse and numerous intraepithelial microcysts, and mild geographic and linear subepithelial opacities are observed. (D): IVCM image shows intraepithelial microcysts with well-defined edges and hyperreflective materials (arrows) inside and outside of them. (E): Tortuous nerves, dendritic cells, and atrophy of Bowman's layer. (F): Active keratocytes present in the deeper stroma.
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case_images: The slit lamp examination revealed multiple diffuse intraepithelial microcysts in the central interpalpebral area and whitish opacities in both corneas (Figure 2). The presence of tortuous subepithelial nerves, dendritic cells, and atrophy of Bowman's layer was frequently observed with active keratocytes in the deeper stroma (Figure 2).
case_images: AS-OCT images of the proband's right eye (patient II-1). (A,B) Images show diffuse hyperreflectivity in the corneal. (A): the epithelial map before MEDC diagnosis, when the patient was wearing contact lenses. (B): 8 months after stopping contact lens use, the epithelial map is thickened in the central and inferotemporal areas.
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case_images: However, pachymetry displayed a diffuse slight thinning pattern (Figure 3).
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case_images: Representative biomicroscopy and IVCM images of the patient's (I-1) left eye. (A-C) illustrate small and numerous microcysts, whitish corneal opacities, gray lines, and positive-fluorescein punctate staining. (D-F): IVCM images. Yellow asterisks indicate microcysts and white arrows indicate epithelial breaks.
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case_images: Slit lamp examination revealed diffuse central intraepithelial corneal microcysts, whitish corneal opacities, gray lines, and positive-fluorescein punctate staining (Figure 4). Tortuous nerves were observed in the subbasal plexus and the stromal layer, demonstrating extensive Bowman's layer alteration and keratocyte activation (Figure 4).
case_images: AS-OCT images of the patient's (I-1) left eye. AS-OCT showed diffuse hyperreflectivity in the corneal epithelium and Bowman's layer. The epithelial map revealed an increase in thickness at the visual axis and mid periphery, possibly due to chronic inflammation.
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case_images: The mean epithelial central thickness was 70 (OD)/74 (OI) microm and the superior and inferior areas showed a clear thinning with values ranging from 39 to 46 microm in both eyes (Figure 5). The corneal thickness in patient I-1 was slightly thinning (Figure 5).