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( Ahreum Song ),( Dae San Yoo ),( Mi Yeon Cho ),( Jong Hoon Kim ),( Soo-chan Kim ) 대한피부과학회 2019 대한피부과학회 학술발표대회집 Vol.71 No.1
IgA mucous membrane pemphigoid (MMP) shares the same clinical feature with MMP; blistering of any or all mucous membranes. Blisters and erosions arise most commonly on oral mucosa followed by ocular, nasal, and genital areas. Histopathological studies demonstrate a subepithelial blister with leukocyte infiltration. The direct immunofluorescence (DIF) reveals a continuous IgA deposition along the basement membrane zone (BMZ), which distinguishes IgA MMP from common MMPs. Also, the indirect immunofluorescence (IIF) often confirms the circulation of IgA autoantibodies in the patients. Herein, we describe a case of IgA MMP in a 60-year-old Korean woman. The patient presented with a 2-year history of ulcerations and erosive lesions of oral mucosa and lips. The skin biopsy revealed subepidermal cleft, spongiosis with exocytosis, and dermal neutrophil and eosinophil infiltrations. The DIF showed a linear IgA deposition along the BMZ, and in the IIF, patient’s IgA reacted on the epidermal side. No positive reactions of BMZ antigen were detected by immunoblotting and ELISA of BP180 and BP230 for both IgG and IgA antibodies; therefore it was unable to distinguish between IgA type MMP and linear IgA dermatosis (LAD). Ultimately, since the typical skin lesions of LAD were absent while the bullous lesions were strictly limited to oral mucosae, we have diagnosed this patient as IgA MMP. The patient showed a good response to methylprednisolone alone.
( Ahreum Song ),( Jieun Jang ),( Soo-chan Kim ),( Jaeyong Shin ),( Jong Hoon Kim ) 대한피부과학회 2021 대한피부과학회 학술발표대회집 Vol.73 No.-
Background: The use of rituximab has been shown to be effective in the treatment of pemphigus, thereby inducing remission efficiently and permitting rapid tapering of corticosteroid doses. The high cost had been a burden in South Korea until Feb. 2018, when the National Health Insurance has started to reimburse the rituximab therapy for patients with severe pemphigus. Objectives: To assess the clinical impact of rituximab reimbursement policy on treatment of pemphigus in South Korea. Methods: A single-center, observational study was conducted including 214 patients with pemphigus treated with their first rituximab from Jan. 2014 to Dec. 2020. T-tests and time series graph comparisons were carried out to evaluate the time of starting rituximab, time taken to achieve the partial remission under minimum therapy (PRMT), and the amount of systemic corticosteroid intake. Results: The time to initiate rituximab was significantly shorter after the rituximab reimbursement policy (p = 0.008). Total systemic corticosteroid intake for the first year of treatment (p = 0.001), and for the 6 months after the rituximab treatment (p = 0.044) were significantly decreased after the rituximab reimbursement policy, but the time to reach the PRMT was not (p = 0.184). Conclusion: The reimbursement policy of rituximab in South Korea had a significant effect on the shortening of time to start rituximab therapy, thereby decrease the amount of total steroid intake after rituximab for the pemphigus patients.
( Ahreum Song ),( Jieun Jang ),( Soo-chan Kim ),( Jaeyong Shin ),( Jong Hoon Kim ) 대한피부과학회 2021 대한피부과학회 학술발표대회집 Vol.73 No.1
Background: The use of rituximab has been shown to be effective in the treatment of pemphigus, thereby inducing remission efficiently and permitting rapid tapering of corticosteroid doses. The high cost had been a burden in South Korea until Feb. 2018, when the National Health Insurance has started to reimburse the rituximab therapy for patients with severe pemphigus. Objectives: To assess the clinical impact of rituximab reimbursement policy on treatment of pemphigus in South Korea. Methods: A single-center, observational study was conducted including 214 patients with pemphigus treated with their first rituximab from Jan. 2014 to Dec. 2020. T-tests and time series graph comparisons were carried out to evaluate the time of starting rituximab, time taken to achieve the partial remission under minimum therapy (PRMT), and the amount of systemic corticosteroid intake. Results: The time to initiate rituximab was significantly shorter after the rituximab reimbursement policy (p = 0.008). Total systemic corticosteroid intake for the first year of treatment (p = 0.001), and for the 6 months after the rituximab treatment (p = 0.044) were significantly decreased after the rituximab reimbursement policy, but the time to reach the PRMT was not (p = 0.184). Conclusion: The reimbursement policy of rituximab in South Korea had a significant effect on the shortening of time to start rituximab therapy, thereby decrease the amount of total steroid intake after rituximab for the pemphigus patients.