Fontaine Juliette, Joly Pascal, Roujeau Jean-Claude
Department of Dermatology of Hôpital Henri Mondor, Université Paris XII, Créteil, France.
J Dermatol. 2003 Feb;30(2):83-90. doi: 10.1111/j.1346-8138.2003.tb00353.x.
Bullous pemphigoid (BP) is the most frequent auto-immune blistering skin disease. Up to recently, it was treated with oral corticosteroids. High dose steroids are poorly tolerated in the elderly and probably contributed to the high mortality rates observed in several cohorts. For years, efforts have been devoted to looking for steroid sparing agents including immunosuppressive drugs, plasma exchanges, intravenous immunoglobulins, tetracycline. Many seemed useful in open series but proved ineffective or marginally effective when tested in randomized controlled trials. An important breakthrough was the demonstration by a large randomized trial that a "super-potent" topical corticosteroid (clobetasol propionate) was not only associated with a significant decrease in severe complications and mortality of BP patients but was also more effective than oral prednisone. New strategies for BP should include topical clobetasol propionate as the first line treatment and consider adjuvant therapy only in the very rare cases that are either resistant to or intolerant of this treatment.
大疱性类天疱疮（BP）是最常见的自身免疫性水疱性皮肤病。直到最近，它一直用口服糖皮质激素治疗。高剂量激素在老年人中耐受性差，可能是导致几个队列中观察到的高死亡率的原因。多年来，人们致力于寻找包括免疫抑制药物、血浆置换、静脉注射免疫球蛋白、四环素在内的激素节省剂。许多药物在开放系列研究中似乎有用，但在随机对照试验中测试时被证明无效或效果甚微。一项重要的突破是一项大型随机试验表明，一种“超强效”外用糖皮质激素（丙酸氯倍他索）不仅与BP患者严重并发症和死亡率的显著降低相关，而且比口服泼尼松更有效。BP的新策略应包括将丙酸氯倍他索作为一线治疗药物，仅在极少数对该治疗耐药或不耐受的情况下考虑辅助治疗。
