Didona Dario, Paolino Giovanni, Di Zenzo Giovanni, Didona Biagio, Pampena Riccardo, Di Nicola Matteo Riccardo, Mercuri Santo Raffaele
Department of Dermatology and Allergology, Philipps University, Marburg, Germany.
Unit of Dermatology, IRCCS San Raffaele Hospital, Milan, Italy.
Dermatol Pract Concept. 2022 Jan 1;12(1):e2022037. doi: 10.5826/dpc.1201a37. eCollection 2022 Feb.
Pemphigus vulgaris (PV) belongs to the group of autoimmune blistering diseases. PV can affect not only mucous membranes, but also the skin and it is characterized by serum IgG autoantibodies against desmoglein 1 and 3, two major components of desmosomes. The introduction of glucocorticoids improved dramatically the prognosis of patients affected by PV. However, long-term use of high dose corticosteroids and adjuvant steroid-sparing immunosuppressants can lead to several adverse events. Rituximab, a chimeric anti-CD20 monoclonal antibody, has been recently approved as in-label therapy for PV, leading to an improvement of the prognosis and higher remission rate. Furthermore, other anti B-cell therapies and several anti-CD20 biosimilars have been introduced in the clinical practice. We focused on present and future therapeutic approaches in PV.
寻常型天疱疮(PV)属于自身免疫性水疱病组。PV不仅可累及黏膜,还可累及皮肤,其特征是血清中存在针对桥粒芯糖蛋白1和3(桥粒的两个主要成分)的IgG自身抗体。糖皮质激素的应用显著改善了PV患者的预后。然而,长期使用高剂量皮质类固醇和辅助性类固醇节省免疫抑制剂会导致多种不良事件。利妥昔单抗,一种嵌合抗CD20单克隆抗体,最近已被批准作为PV的标记内治疗药物,从而改善了预后并提高了缓解率。此外,其他抗B细胞疗法和几种抗CD20生物类似药已被引入临床实践。我们关注PV目前和未来的治疗方法。