Dermatology and Venereology Department, Hospital Universitario 12 de Octubre, Universidad Complutense de Madrid, Madrid, Spain.
Dermatol Ther. 2019 Mar;32(2):e12829. doi: 10.1111/dth.12829. Epub 2019 Feb 7.
Bullous pemphigoid (BP) is an acquired autoimmune bullous disease characterized by autoantibodies against the hemidesmosomal proteins found in the basal keratinocytes of the basement membrane zone (BMZ): a 180 kDa protein (type XVII collagen) mainly and the 230 kDa antigen. There is such evidence that the antibodies against the BMZ components are not only of IgG type, but also this bullous disease may have IgE antibodies directed to the BMZ that contribute to the pathogenesis of the disorder. IgE is not only thought to contribute to the pathogenesis of BP, it has also been suggested that eosinophils play a role in the development of the first signs associated with BP. A humanized monoclonal antibody directed to IgE, omalizumab, is approved for the treatment of severe asthma and chronic spontaneous urticaria, and it may be useful in the treatment of BP in the first stages of the disease.
大疱性类天疱疮(BP)是一种获得性自身免疫性大疱性疾病,其特征是自身抗体针对基底膜带(BMZ)中基底角蛋白细胞中的半桥粒蛋白:主要是 180kDa 蛋白(XVII 型胶原)和 230kDa 抗原。有证据表明,针对 BMZ 成分的抗体不仅是 IgG 类型,而且这种大疱性疾病可能还具有针对 BMZ 的 IgE 抗体,这有助于发病机制。IgE 不仅被认为有助于 BP 的发病机制,而且还表明嗜酸性粒细胞在与 BP 相关的早期迹象的发展中发挥作用。针对 IgE 的人源化单克隆抗体奥马珠单抗已被批准用于治疗严重哮喘和慢性自发性荨麻疹,它在疾病的早期阶段治疗 BP 可能有用。