RESUMEN
Objective:To investigate the efficacy and safety of minocycline alone or in combination with low-dose glucocorticoids in the treatment of pemphigus erythematosus and pemphigus herpetiformis, as well as their effect on immune indices.Methods:A retrospective study was conducted, and patients with newly diagnosed pemphigus erythematosus or pemphigus herpetiformis, who received initial treatment with minocycline alone or in combination with low-dose glucocorticoids and were followed up for more than 6 months, were collected from Department of Dermatology, Peking University First Hospital from June 2011 to June 2021. Data on patients′ condition and autoantibody levels were collected at baseline and different follow-up time points, and disease severity, diagnosis, changes in autoantibody levels and their relationships with efficacy were analyzed. The Kaplan-Meier method was used to analyze complete remission rate, and chi-square test to analyze the efficacy among patients with different disease severity or after different treatments.Results:A total of 24 patients of Han nationality were collected and followed up for a median period of 21.8 months, including 15 with pemphigus erythematosus and 9 with pemphigus herpetiformis. The male to female ratio was 1.4∶1, their median age was 68.8 years, and the median duration of disease was 22.1 months. All the 24 patients achieved disease control, and the time to disease control ( M [ Q1, Q3]) was 15.9 (12, 20.1) weeks. Twenty-three (95.8%) patients achieved complete remission, and the time to complete remission was 8.7 (6.4, 10) months. After 1-year treatment, no significant difference in the complete remission rate was observed between patients receiving minocycline monotherapy (11/13) and those receiving combination therapy with low-dose glucocorticoids (9/11, χ2 = 0.16, P = 0.692) . During the follow-up period, 2 patients (8.7%) experienced recurrence in disease control state, 1 of whom achieved complete remission at week 38 after dose adjustment, and the other achieved complete remission after half-a-year treatment with rituximab. There was no significant difference in the efficacy between patients with mild and moderate pemphigus ( χ2 = 0.28, P = 0.599) . Drug-related adverse reactions occurred in 3 cases, including 1 case of tinea corporis on the back and 2 cases of generalized hyperpigmentation of the skin and gingiva. Conclusion:Minocycline alone or in combination with low-dose glucocorticoids was effective for the treatment of mild to moderate pemphigus erythematosus and pemphigus herpetiformis without serious adverse reactions, but long-term efficacy, adverse reactions and patients′ prognosis should be re-evaluated in prospective multi-center studies with a large sample size in the future.
RESUMEN
Objective:To retrospectively analyze the efficacy and safety of dupilumab in the treatment of bullous pemphigoid (BP) .Methods:Clinical data were collected from BP patients who received injections of dupilumab at an initial dose of 600 mg followed by an every-2-week regimen at a dose of 300 mg (the frequency of injections could be increased if necessary) in Department of Dermatology, Peking University First Hospital from October 2020 to October 2021, and their clinical manifestations and changes in laboratory indices were analyzed.Results:A total of 21 BP patients treated with dupilumab were included in this study. Nineteen (90.5%) patients achieved complete or marked disease control after 2-week treatment with dupilumab; 12 patients were followed up for 16 weeks, and all maintained complete disease control at 16 weeks. All patients had a bullous pemphigoid disease area index (BPDAI) score of 122.5 ± 51.1 points at baseline, which decreased to 30.6 ± 27.4 points after 2-week treatment with dupilumab ( t = 8.53, P < 0.001) , and continued to decrease to 12.7 ± 9.1 points after 4-week treatment ( t = 9.73, P < 0.001) . Pruritus was markedly relieved in all the 21 patients within 4-week treatment with dupilumab. Among 10 patients with elevated eosinophil counts at baseline, the eosinophil counts markedly decreased in 9 after treatment. The serum IgE level was elevated in 7 patients at baseline, which markedly decreased in 6 after treatment. Viral conjunctivitis occurred in 1 (4.8%) patient, and no adverse reactions were observed in other patients. Conclusion:Dupilumab is effective in the control of BP and relief of pruritus, with a favorable safety profile.