case_images: PMC3806711_01
case_images: 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.
case_images: cop-0004-0114-g01.jpg
case_images: PMC3806711_01_cop-0004-0114-g01.jpg
case_images: F1
case_images: 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).
case_images: 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.
case_images: cop-0004-0114-g02.jpg
case_images: PMC3806711_01_cop-0004-0114-g02.jpg
case_images: F2
case_images: 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).
case_images: 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.
case_images: cop-0004-0114-g03.jpg
case_images: PMC3806711_01_cop-0004-0114-g03.jpg
case_images: F3
case_images: 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).