Department of Dermatology and Venereology, Zhuji People's Hospital, Wenzhou Medical University, Zhuji, China.
Department of Dermatology and Venereology, Sir Run Run Shaw Hospital, Zhejiang University School of Medicine, Hangzhou, China.
Hum Vaccin Immunother. 2023 Dec 31;19(1):2151290. doi: 10.1080/21645515.2022.2151290. Epub 2023 Feb 16.
Pemphigus is a rare autoimmune disease and has the potential to be fatal without treatment. Pemphigus erythematosus (PE) is a benign type of pemphigus foliaceus. Glucocorticoids and immunosuppressive agents are primary therapeutic modalities in pemphigus erythematosus, which may lead to considerable side effects. There is a growing need for new pemphigus therapies with fewer adverse effects. Dupilumab is a humanized monoclonal IgG4 antibody that inhibits the signaling of interleukin-4 (IL-4) and interleukin-13 (IL-13) and has been applied for atopic dermatitis and asthma. Recently, dupilumab was thought to be beneficial in aggressive refractory pemphigus vulgaris. We report two cases: a 39-year-old male and a 59-year-old woman diagnosed with PE with atypical clinical features. With dupilumab, patients' skin lesions significantly improved, and suitable maintenance glucocorticosteroid doses were reached. In conclusion, we reported the short-term effectiveness and safety of dupilumab in two cases of atypical generalized PE. As an adjunct, such a biologic agent is expected to be efficacious in pemphigus erythematosus.
天疱疮是一种罕见的自身免疫性疾病，如果不治疗，可能致命。红斑性天疱疮 (PE) 是一种良性的落叶型天疱疮。糖皮质激素和免疫抑制剂是治疗红斑性天疱疮的主要方法，但可能会导致相当多的副作用。人们越来越需要副作用更少的新型天疱疮治疗方法。度普利尤单抗是一种人源化单克隆 IgG4 抗体，可抑制白细胞介素-4 (IL-4) 和白细胞介素-13 (IL-13) 的信号传导，已应用于特应性皮炎和哮喘。最近，度普利尤单抗被认为对侵袭性难治性寻常型天疱疮有益。我们报告了两例病例：一例 39 岁男性和一例 59 岁女性，诊断为具有非典型临床特征的 PE。使用度普利尤单抗后，患者的皮肤病变明显改善，并达到了合适的维持糖皮质激素剂量。总之，我们报告了度普利尤单抗在两例非典型全身性 PE 中的短期疗效和安全性。作为一种辅助药物，这种生物制剂有望对红斑性天疱疮有效。
