Dick Sarah E, Werth Victoria P
Department of Dermatology, Philadelphia VA Medical Center, University of Pennsylvania, Philadelphia, PA, USA.
Autoimmunity. 2006 Nov;39(7):591-9. doi: 10.1080/08916930600972008.
Pemphigus is a group of rare autoimmune mucocutaneous bullous diseases with potential significant morbidity and mortality. The two main subtypes are pemphigus vulgaris (PV) and pemphigus foliaceus (PF). Systemic corticosteroid use and other advances in management have dramatically decreased the mortality rate for pemphigus. At present, the primary cause of morbidity and mortality is complications from treatment. Thus, the goal of pemphigus management is to induce and maintain remission with the lowest possible doses of medication and with the fewest side effects. Although our scientific knowledge of pemphigus is advancing and our treatment options are expanding, there are still very few randomized, controlled studies to evaluate the true effectiveness of the available therapies. Here we review the available treatment options and novel therapies for pemphigus and the supporting data.
天疱疮是一组罕见的自身免疫性黏膜皮肤大疱性疾病,具有潜在的显著发病率和死亡率。两个主要亚型是寻常型天疱疮(PV)和落叶型天疱疮(PF)。全身使用皮质类固醇及其他治疗进展已显著降低了天疱疮的死亡率。目前,发病和死亡的主要原因是治疗并发症。因此,天疱疮治疗的目标是以尽可能低的药物剂量和最少的副作用诱导并维持缓解。尽管我们对天疱疮的科学认识在不断进步,治疗选择也在不断扩展,但仍很少有随机对照研究来评估现有疗法的真正有效性。在此,我们综述天疱疮的现有治疗选择、新疗法及相关支持数据。