Figure 3. In vivo confocal microscopy images of representative affected family members. The right eye (virgin cornea) of the proband (frame size 400 µm x 400 µm) at the level of (A) basal epithelial layer illustrating focal deposition of homogeneous, reflective materials with rounded edges, and hyporeflective borders and (B) posterior stroma showing extensive scarring with arrow indicating “ghost” blood vessels. C: Corresponding slit-lamp biomicroscopy photograph illustrating dense scarring. D: In vivo confocal microscopy of the allograft in the right eye of subject II:1 at the level of Bowman’s layer which has been completely replaced by diffuse, homogeneous, reflective material (frame size 400 µm x 400 µm). E: Corresponding slit-lamp biomicroscopy photograph illustrating recurrence of the dystrophy within the peripheral right corneal graft of subject II:1, 4 years following penetrating keratoplasty.