PMC5633293_01
(A) and (B) show generalized diffuse scleritis with scleral abscess inferotemporal area.
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In intraoperative, we found scleral necrosis with small abscess (Figure 1A and B) at inferotemporal area.
Turbid yellowish vitreous fluid from diagnostic vitrectomy.
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The fundus showed dense yellowish turbid vitritis (Figure 2), retinal and perivascular infiltration, retinal edema, and shallow subretinal fluid at inferior retina.
A scleral tissue biopsy showing mucosal erosion and submucosal fibrosis with necrobiosis of collagen and sheets of mononucleated foamy histiocytes (xanthoma cells), infiltrating the submucosal fibrous tissue, accompanied by variable numbers of dispersed lymphocytes and plasma cells (hematoxylin-eosin; original magnification: x40).
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Multiple scleral biopsies were performed, and the findings revealed erosive scleral mucosa with submucosal infiltration by sheets of mononucleated foamy histiocytes (xanthoma cells), accompanied by variable degree of fibrosis with necrobiosis of collagen (Figures 3 and 4).
Another scleral tissue biopsy predominantly containing fibrosis with necrobiosis of collagen and scattered necrotic cellular debris of inflammatory cells (hematoxylin-eosin; original magnification: x200).
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Multiple scleral biopsies were performed, and the findings revealed erosive scleral mucosa with submucosal infiltration by sheets of mononucleated foamy histiocytes (xanthoma cells), accompanied by variable degree of fibrosis with necrobiosis of collagen (Figures 3 and 4).
Sheets of xanthoma cells and scattered hemosiderin- laden macrophages, neutrophils, lymphocytes, plasma cells, and erythrocytes. The xanthoma cells have small, round nuclei and abundant clear or vacuolated cytoplasm (hematoxylin-eosin; original magnification: x400).
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Touton giant cells, or cholesterol clefts were scattered among these xanthoma cells. These infiltrating xanthoma cells often have small, round nuclei, and abundant clear or vacuolated cytoplasm (Figure 5).
Some of infiltrating histiocytes show hemophagocytic activity characterized by intracytoplasmic nuclear debris (hematoxylin-eosin; original magnification: x400).
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Some of these histiocytes show hemophagocytic activity (Figure 6).
Small- and medium-sized vasculitis characterized by lymphocytic and neutrophilic infiltrate in wall of subcutaneous vessels obtained from sural nerve biopsy (hematoxylin-eosin; original magnification: x200).
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Left sural nerve biopsy confirmed small- and medium-sized vasculitis by lymphocytic and neutrophilic infiltrate in wall of subcutaneous vessels (Figure 7).