Bullous pemphigoid
Bullous pemphigoid
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DOI：
10.1590/abd1806-4841.20199007
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发表时间：
2019-04-01
影响因子：
1.7
通讯作者：
Maruta, Celina Wakisaka
中科院分区：
文献类型：
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作者：
Miyamoto, Denise;Santi, Claudia Giuli;Maruta, Celina Wakisaka
大疱性类天疱疮是最常见的自身免疫性大疱性疾病，主要影响老年人。在过去的几十年中，发病率的增加归因于人口老龄化，药物诱导的病例和疾病的非大疱性表现的诊断的改善。失调的T细胞免疫应答和针对半桥粒蛋白（BP 180和BP 230）的IgG和IgE自身抗体的合成导致嗜中性粒细胞趋化性和基底膜区的降解。大疱性类天疱疮的典型表现为躯干和四肢荨麻疹斑块上的紧张性水疱，伴有强烈瘙痒。很少报告粘液瘤受累。诊断依赖于（1）组织病理学评价，证明嗜酸性海绵状组织增生或嗜酸性粒细胞表皮下脱离;（2）使用直接或间接免疫荧光测定法检测基底膜区的IgG和/或C3沉积;和（3）使用ELISA定量针对BP 180和/或BP 230的循环自身抗体。大疱性类天疱疮通常与老年人的多种合并症相关，特别是神经系统疾病和血栓形成风险增加，1年死亡率达到23%。治疗必须根据患者的临床状况和疾病严重程度进行量身定制。高效外用类固醇和全身性类固醇是目前治疗的主流。最近的随机对照研究已经证明了多西环素、氨苯砜和免疫抑制剂辅助治疗的益处和安全性，目的是减少累积类固醇剂量和死亡率。
Bullous pemphigoid is the most frequent autoimmune bullous disease and mainly affects elderly individuals. Increase in incidence rates in the past decades has been attributed to population aging, drug-induced cases and improvement in the diagnosis of the nonbullous presentations of the disease. A dysregulated T cell immune response and synthesis of IgG and IgE autoantibodies against hemidesmosomal proteins (BP180 and BP230) lead to neutrophil chemotaxis and degradation of the basement membrane zone. Bullous pemphigoid classically manifests with tense blisters over urticarial plaques on the trunk and extremities accompanied by intense pruritus. Mucosal involvement is rarely reported. Diagnosis relies on (1) the histopathological evaluation demonstrating eosinophilic spongiosis or a subepidermal detachment with eosinophils; (2) the detection of IgG and/or C3 deposition at the basement membrane zone using direct or indirect immunofluorescence assays; and (3) quantification of circulating autoantibodies against BP180 and/or BP230 using ELISA. Bullous pemphigoid is often associated with multiple comorbidities in elderly individuals, especially neurological disorders and increased thrombotic risk, reaching a 1-year mortality rate of 23%. Treatment has to be tailored according to the patient's clinical conditions and disease severity. High potency topical steroids and systemic steroids are the current mainstay of therapy. Recent randomized controlled studies have demonstrated the benefit and safety of adjuvant treatment with doxycycline, dapsone and immunosuppressants aiming a reduction in the cumulative steroid dose and mortality.