Department of Dermatology, King's College Hospital, London, UK.
J Oral Pathol Med. 2011 Sep;40(8):616-20. doi: 10.1111/j.1600-0714.2011.01017.x. Epub 2011 Mar 9.
Pemphigus vulgaris (PV) is a life-threatening autoimmune blistering disease affecting the skin and mucosa and is associated with increased morbidity and mortality. Once the diagnosis is established, the main stay of treatment of PV is with systemic corticosteroids to control the disease and then to consolidate the management with other immunosuppressive agents. A small group of patients with severe pemphigus, however, remain relcalcitrant to both steroids and azathioprine, and disease is difficult to control. When considering the treatment of refractory PV, there is increasing evidence for the successful use of the monoclonal anti-CD20 antibody, rituximab. We report here six cases of patients presenting with oral and skin PV with recalcitrant or rapidly progressive disease treated with a novel dosing regimen of rituximab as a single agent. All patients achieved a complete response to a maximum follow-up of 34 months.
寻常型天疱疮（PV）是一种危及生命的自身免疫性水疱病，影响皮肤和黏膜，与发病率和死亡率增加有关。一旦确诊，PV 的主要治疗方法是使用全身性皮质类固醇来控制疾病，然后用其他免疫抑制剂来巩固治疗。然而，一小部分严重的天疱疮患者对类固醇和硫唑嘌呤均有抗药性，且疾病难以控制。在考虑难治性 PV 的治疗时，越来越多的证据表明使用单克隆抗 CD20 抗体利妥昔单抗是成功的。我们在此报告 6 例口腔和皮肤均有天疱疮且疾病顽固或快速进展的患者，他们接受了利妥昔单抗作为单一药物的新型剂量方案治疗。所有患者在最长 34 个月的随访中均达到完全缓解。
