case_images: PMC9198329_01
case_images: Changes of clinical features of this patient. The clinical features on the gingivae (left) and buccal mucosa (right) at Days 0, 30 and 50 are shown.
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case_images: Clinically, erosive oral mucosal lesions improved with time on the therapy (Figure 1, Day 30 and Day 50).
case_images: Histopathological features of this patient. Histopathological features for the biopsy taken from the lesions on the left buccal mucosa (H&E staining, original magnification, x200). Blue, green and yellow arrows indicated the vacuolar degeneration, plasma cell and lymphocyte, respectively.
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case_images: Day 0) without any lesions on the skin or other mucous membranes Histopathology of biopsy from white striae on the left buccal mucosa showed atrophic mucosal epithelium with vacuolar degeneration of basal cells, and severe inflammatory infiltration of lymphocytes and plasma cells with formation of lymphoid follicle in the upper dermis (Figure 2). Direct immunofluorescence showed no BMZ deposition for IgG, IgA, IgM and C3 (data not shown).
case_images: Indirect immunofluorescence using normal human skin (IIF) and using 1M NaCl-split normal human skin (ssIIF) for the patient serum taken at Day 0 and Day 50. IgA (A), but not IgG of (B), anti-BMZ antibodies were detected in IIF for serum taken at Day 0. IgA (C), but not IgG (D) antibodies bound to the dermal side of the split in ssIIF for serum taken at Day 0. IgA antibodies were not detected in both IIF (E) and ssIIF (F) for serum taken at Day 50.
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case_images: B). By IIF using 1M NaCl-split normal human skin (ssIIF), the patient serum taken at Day 0 showed only IgA binding to the dermal side of the split skin (Figures 3C, D). IgA anti-BMZ antibodies were detectable for sera collected at Day 30 and Day 50 (Figures 3E, F and data not shown). In immunoblotting of normal human dermal extract, IgA reactivity with LMgamma1 reduced gradually with time (Figure 4A). In immunoblotting analyses of rLM111 and rLM521, IgG reactivities with LMgamma1 were not changed with time (Figures 4B, C). In immunoblotting of rLM521, IgA reactivity with LMalpha5 at Day 30 was similar to that at Day 0, but reduced at Day 50 (Figure 4C).
case_images: Immunoblotting analyses. Immunoblotting analyses of normal human dermal extract (A), rLM111 (B) and rLM521 (C) were performed for IgG and IgA autoantibodies against LMgamma1 and LMalpha5 in patient sera collected at Days 0, 30 and 50. The serum of an anti-LMgamma1 pemphigoid patient and anti-LMalpha5 monoclonal antibody (LMalpha5 mAb) were used as positive controls.
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case_images: IgA, but not IgG, autoantibodies against LMgamma1 (Figure 4A). Immunoblotting of LM111 recombinant protein (rLM111) detected IgG, but not IgA, autoantibodies against LMgamma1 (Figure 4B). Furthermore, IgA anti-LMalpha5 and IgG anti-LMgamma1 autoantibodies were identified in immunoblotting of rLM521 (Figure 4C). IgA anti-BMZ antibodies were detectable for sera collected at Day 30 and Day 50 (Figures 3E, F and data not shown). In immunoblotting of normal human dermal extract, IgA reactivity with LMgamma1 reduced gradually with time (Figure 4A). In immunoblotting analyses of rLM111 and rLM521, IgG reactivities with LMgamma1 were not changed with time (Figures 4B, C). In immunoblotting of rLM521, IgA reactivity with LMalpha5 at Day 30 was similar to that at Day 0, but reduced at Day 50 (Figure 4C).