a Unit of Dermatology , University of Padua , Padua , Italy.
Expert Opin Biol Ther. 2019 Feb;19(2):149-156. doi: 10.1080/14712598.2019.1559291. Epub 2018 Dec 18.
Autoimmune blistering skin diseases are a group of disorders subdivided according to the location of blister formation: intraepidermal blistering in the pemphigus group and subepidermal in the pemphigoid group. These conditions are clinically heterogeneous and are treated with systemic corticosteroids and/or other forms of immunosuppression on the basis of clinical subtype and disease severity. These approaches may not be effective for the induction and maintenance of clinical response or need to be stopped because of intolerable side effects.
Biological therapies can represent a valid alternative strategy in various autoimmune blistering disorders and this review article will address this issue with a special focus on pemphigus vulgaris and bullous pemphigoid. These biological approaches are designed to target B cells, autoantibodies, complement proteins, and several cytokines.
Innovative strategies for the treatment of autoimmune blistering conditions primarily depend on the use of drugs with a high degree of specificity targeting crucial steps in the immunopathology of these disorders. Novel biological agents offer treatment alternatives to patients with autoimmune blistering conditions by targeting B cells, pathogenic autoantibodies, complement and cytokines.
自身免疫性水疱性皮肤病是一组根据水疱形成部位进行细分的疾病：棘层松解性大疱性皮肤病（天疱疮）为表皮内水疱性疾病，基底膜带松解性大疱性皮肤病（类天疱疮）为表皮下水疱性疾病。这些疾病在临床上具有异质性，根据临床亚型和疾病严重程度，采用全身性皮质类固醇和/或其他形式的免疫抑制治疗。这些方法可能无法有效诱导和维持临床反应，或者因为无法耐受副作用而需要停药。
生物疗法在各种自身免疫性水疱性疾病中可能是一种有效的替代策略，本文将特别关注寻常型天疱疮和大疱性类天疱疮。这些生物疗法旨在针对 B 细胞、自身抗体、补体蛋白和多种细胞因子。
治疗自身免疫性水疱性疾病的创新策略主要取决于使用针对这些疾病免疫病理学关键步骤的高度特异性药物。新型生物制剂通过针对 B 细胞、致病性自身抗体、补体和细胞因子，为自身免疫性水疱性疾病患者提供了治疗选择。
