关键词: 肿瘤坏死因子α, 核因子- kappa B, p38丝裂原活化蛋白激酶, HB-13, HP-13, 类天疱疮,大疱性, 糖皮质激素类, 免疫抑制剂

Abstract: Objective To summarize our experience of treating 124 patients with bullous pemphigoid(BP)and propose a treatment algorithm based on the affected body surface area(BSA). Methods A retrospective study was performed of 124 patients with a diagnosis of bullous pemphigoid admitted to Beijing University First Hospital over a period of 20 years. The initial dose of prednisone was 20-30mg/d, 40 mg/d, or 60 mg/d, based on baseline affected BSA of less than 10%, 10-50%, or greater than 50%, respectively. If adequate response was not achieved within one week, the dose was increased by 50% up to 100 mg/L. Pulsed intravenous methylprednisolone 250 or 500 mg/d for 3 days was given to refractory cases. Meanwhile, a concomitant immunosuppressant was given to most moderate-to-severe cases within the first 8 weeks of treatment or to control the mild recurrence during maintenance therapy. Such induction therapy continued until resolution of skin lesions, followed by gradual tapering of dose to discontinue therapy in several months to years. Results Of these patients, 91 experienced an outcome of "clinical cure" or "almost cure",12 died during the follow-up period, 4 lost to follow-up,the other 17 were still on maintenance therapy. Conclusion The algorithm of combination therapy with corticosteroids and other immunosuppressants based on the affected BSA was proved to be effective and practical to treat BP.

Key words: Pemphigoid, bullous, Glucocorticoids, Immunosuppressive agents