BACKGROUND: Pemphigus vulgaris (PV) is an autoimmune disease characterized by acantholysis in the epithelium. PV is often difficult to diagnose in the early stages, since the oral lesions are relatively nonspecific. We report on a case with a favorable outcome due to early diagnosis and effective treatment of oral lesions. METHODS: A 53-year-old Japanese woman presented with a month-long history of painful gingiva. PV was diagnosed using clinical, histopathological, and direct immunofluorescence criteria 1 month after the first visit. Evaluation of the circulating autoantibody titers to desmoglein (Dsg)1 and Dsg3 was conducted by enzyme-linked immunosorbent assay (ELISA). Occlusive steroid therapy (OST) using a topical corticosteroid (0.1% triamcinolone acetonide) was provided for 8 weeks. After OST was completed, a buccal frenectomy was performed to eliminate localized toothbrushing trauma. RESULTS: There were no adverse effects on wound healing after the procedure. No oral PV lesions were detectable at the 9-month reevaluation, and the patient remains in complete remission. The Dsg1 ELISA was negative while the Dsg3 ELISA was indeterminate at the first visit. The Dsg1 ELISA was also negative at reevaluation, but Dsg3 ELISA reactivity had increased 9 months after completion of treatment. CONCLUSION: Large scale clinical trials will be required to validate the clinical benefits of the OST treatment regimen, and further research is necessary to determine the importance of ELISA antibody examination in long-term management of oral PV.
BACKGROUND: Pemphigus vulgaris (PV) is an autoimmune disease characterized by acantholysis in the epithelium. PV is often difficult to diagnose in the early stages, since the oral lesions are relatively nonspecific. We report on a case with a favorable outcome due to early diagnosis and effective treatment of oral lesions. METHODS: A 53-year-old Japanese woman presented with a month-long history of painful gingiva. PV was diagnosed using clinical, histopathological, and direct immunofluorescence criteria 1 month after the first visit. Evaluation of the circulating autoantibody titers to desmoglein (Dsg)1 and Dsg3 was conducted by enzyme-linked immunosorbent assay (ELISA). Occlusive steroid therapy (OST) using a topical corticosteroid (0.1% triamcinolone acetonide) was provided for 8 weeks. After OST was completed, a buccal frenectomy was performed to eliminate localized toothbrushing trauma. RESULTS: There were no adverse effects on wound healing after the procedure. No oral PV lesions were detectable at the 9-month reevaluation, and the patient remains in complete remission. The Dsg1 ELISA was negative while the Dsg3 ELISA was indeterminate at the first visit. The Dsg1 ELISA was also negative at reevaluation, but Dsg3 ELISA reactivity had increased 9 months after completion of treatment. CONCLUSION: Large scale clinical trials will be required to validate the clinical benefits of the OST treatment regimen, and further research is necessary to determine the importance of ELISA antibody examination in long-term management of oral PV.