Background

Bullous pemphigoid (BP) is an autoimmune blistering disorder occurring mostly in the elderly that lacks adequate treatments.

Objective

To describe our experience using dupilumab in a series of patients with BP.

Methods

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.

Results

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.

Limitations

Include small sample size, lack of a control group, lack of a standardized assessment tool, and lack of standardized safety monitoring.

Conclusion

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.

中文翻译：

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Dupilumab作为大疱性类天疱疮的新疗法：多中心病例系列。

背景

大疱性类天疱疮（BP）是一种自身免疫性水疱性疾病，主要发生在缺乏适当治疗的老年人中。

目的

描述我们在一系列BP患者中使用dupilumab的经验。

方法

这是来自5个学术中心接受dupilumab进行BP治疗的病例系列。如果患者通过病变皮肤活检证实患有BP的临床诊断是合格的，则可以通过以下一项或多项评估：苏木精和曙红染色，直接免疫荧光或BP180或BP230的酶联免疫吸附测定，或两者。

结果

我们确定了13例患者。患者平均年龄为76.8岁，在开始dupilumab之前BP的平均持续时间为28.8个月（范围1-60个月）。92.3％（13名患者中的12名）患者实现了疾病清除或满意的反应。令人满意的反应定义为临床医生对疾病改善的记录，以及患者希望在没有疾病清除记录的情况下继续使用药物的意愿。53.8％（13例中的7例）患者的BP总清除率未见报道。

局限性

包括样本量小，缺少对照组，缺乏标准化的评估工具以及缺乏标准化的安全监控。

结论

Dupilumab可能是BP的另一种治疗方法，可导致92.3％的患者（包括先前常规治疗失败的患者）获得疾病清除或满意的反应。