Fig. 2

Histology of enucleated globe from case 1. a At low power, serous detachment with subretinal protein (p), uveitis (u) with choroidal thickening, and scleritis (s) with scleral/episcleral thickening are appreciated. The abrupt defect in the episcleral fibrotic plaque medially corresponds to the prior episcleral/orbital biopsy site (arrowhead). Optic nerve cupping is also evident. The gap in the eye wall where the cornea would ordinarily be (c) is artifactual (H&E stain, original magnification, ×1). b Higher magnification of same region of globe medially that was labeled in a. Besides the previously mentioned findings, episcleral/scleral fibrosis (f) is appreciated surrounding the pockets of inflammation, corresponding to the fibrotic-appearing epsicleral/scleral plaque that was noted clinically (H&E stain, original magnification, ×20). c Higher magnification reveals granulomatous uveitis (u), with several giant cells visible in the field (arrowheads). Sclera (s) is visible at the bottom of the field (H&E stain, original magnification, ×200). d Higher magnification of sclera reveals necrotizing and granulomatous scleritis, with both neutrophils (n) and macrophages including a giant cell (arrowhead) visible in the field (H&E stain, original magnification, ×400). e Higher magnification of optic nerve, revealing peri-neuritis/neuritis, with chronic inflammatory cells infiltrating both the optic nerve sheath (arrows) and pial septa within the optic nerve (arrowheads). Optic nerve cupping/atrophy is also seen (H&E stain, original magnification, ×40). f Higher magnification of the retina reveals severe atrophy and exhibits the chronic vasculitis that was noted in some of the retinal vessels (arrow) (H&E stain, original magnification, ×200). g IgG4 immunostain highlights >70 plasma cells per high-power field, corresponding to >50 % of the plasma cell population, in the uvea and sclera/episclera. The uvea is captured in this field. The double-headed arrow delineates the subretinal space (original magnification, ×400)