PMC3806711_01
Slit lamp photograph, histology, and direct immunofluorescent staining of palpebral conjunctiva. a Right conjunctiva. b Left conjunctiva. c Left conjunctiva in December 2010. The giant papillae resolved with increased systemic treatment. d Resected tissues revealed marked inflammatory reaction. HE. Note epithelial acantholysis and marked inflammatory cell infiltration, including lymphocytes, plasma cells, and neutrophils in the subconjunctival tissue. e, f Direct immunofluorescent staining of IgG (e) and C3 (f) showing the intercellular deposition of each protein in the epithelium.
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F1
Both the eyelids were edematous, and the superior palpebral conjunctivas showed giant cobblestone-like papillae mostly laterally (fig. 1a, b). Marked acantholysis was observed in the epithelium (fig. 1d). Direct immunofluorescent staining showed a significant deposition of IgG and C3 in the epithelial intercellular spaces (fig. 1d, f). The ophthalmic symptoms and conjunctival giant papillae resolved with the increased systemic treatment (fig. 1c).
Histology and immunohistochemical staining in the subconjunctival tissue. a Note marked inflammatory cell infiltration. HE. b Inflammatory cells composed of lymphocytes, plasma cells, and a few neutrophils. HE. c Toluidine blue staining revealed that the subconjunctival tissue infrequently contained mast cells. The inset shows a mast cell with metachromasia (arrow). d C-kit immunostaining reveals only a few mast cells. The inset shows a positive cell (arrow). Immunohistochemical findings of CD4 (e), CD8 (f), CD20 (g), and CD79a (h) in the resected subconjunctival tissue. CD4+ T cells are represented almost equally with CD8+ T cells, and CD79a+ cells predominate over CD20+ cells, indicating plasma cell infiltration.
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F2
Toluidine blue staining and c-kit immunostaining showed minimal mast cells (fig. 2c, d). Immunostaining also showed CD4+ T cells had infiltrated to almost the same degree as CD8+ cells (fig. 2e, f). Immunostaining of CD20 and CD79a indicated numerous mature B cell and plasma cell infiltrations (fig. 2g, h).
Clinical course and index value transitions. Anti-Dsg1 and anti-Dsg3 antibody index values gradually decreased with increased systemic treatment. Dsg1 = Anti-Dsg1 antibody index (values shown in squares); Dsg3 = anti-Dsg3 antibody index (values shown in squares); light grey meshing = prednisolone application (doses shown in brackets; mg/day); arrowheads = intravenous immunoglobulin administration.
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F3
The anti-Dsg1 and anti-Dsg3 antibody index values gradually decreased and were accompanied by oral erosion improvement and the ability to eat (fig. 3).