Department of Dermatology, Tufts Medical Center, Boston, Massachusetts.
Department of Dermatology, University of Connecticut Health Center, Farmington, Connecticut.
J Am Acad Dermatol. 2020 Jul;83(1):46-52. doi: 10.1016/j.jaad.2020.01.089. Epub 2020 Mar 13.
Bullous pemphigoid (BP) is an autoimmune blistering disorder occurring mostly in the elderly that lacks adequate treatments.
To describe our experience using dupilumab in a series of patients with BP.
This is a case series of patients from 5 academic centers receiving dupilumab for BP. Patients were eligible if they had a clinical diagnosis of BP confirmed by lesional skin biopsy evaluated by one of more of the following: hematoxylin and eosin staining, direct immunofluorescence, or enzyme-linked immunosorbent assay for BP180 or BP230, or both.
We identified 13 patients. Patients were an average age of 76.8 years, and the average duration of BP before dupilumab initiation was 28.8 months (range, 1-60 months). Disease clearance or satisfactory response was achieved in 92.3% (12 of 13) of the patients. Satisfactory response was defined as clinician documentation of disease improvement and patient desire to stay on the medication without documentation of disease clearance. Total clearance of the BP was achieved in 53.8% (7of 13) of patients No adverse events were reported.
Include small sample size, lack of a control group, lack of a standardized assessment tool, and lack of standardized safety monitoring.
Dupilumab may be an additional treatment for BP, leading to disease clearance or satisfactory response in 92.3% of patients, including in those in whom previous conventional therapy had failed.
大疱性类天疱疮（BP）是一种主要发生在老年人中的自身免疫性水疱性疾病，缺乏有效的治疗方法。
描述我们在一系列 BP 患者中使用度普利尤单抗的经验。
这是一项来自 5 个学术中心的患者的病例系列研究，这些患者接受度普利尤单抗治疗 BP。如果患者的临床诊断为 BP，并通过以下一种或多种方法证实：苏木精和伊红染色、直接免疫荧光或酶联免疫吸附试验检测 BP180 或 BP230，或两者均阳性，则符合入组条件。
我们共确定了 13 名患者。患者的平均年龄为 76.8 岁，在开始使用度普利尤单抗前，BP 的平均病程为 28.8 个月（范围为 1-60 个月）。92.3%（12/13）的患者达到疾病清除或满意缓解。满意缓解定义为临床医生记录的疾病改善和患者希望继续使用药物而无需记录疾病清除。53.8%（7/13）的患者达到 BP 完全清除。没有报告不良事件。
包括样本量小、缺乏对照组、缺乏标准化评估工具以及缺乏标准化安全性监测。
度普利尤单抗可能是 BP 的一种额外治疗方法，可使 92.3%的患者达到疾病清除或满意缓解，包括那些先前常规治疗失败的患者。
