Engineer L, Bhol K, Kumari S
Department of Oral Medicine and Diagnostic Sciences, Harvard School of Dental Medicine, Boston, MA, USA
Cytokine. 2001 Jan 7;13(1):32-38. doi: 10.1006/cyto.2000.0791.
Bullous pemphigoid (BP) is an autoimmune blistering skin disease characterized by the presence of autoantibodies to normal components of the hemidesmosome (BPAg1 and BPAg2). Histology of skin lesions demonstrate a subepidermal vesicle and a predominantly eosinophilic dermal cellular infiltrate. Interleukin 5 (IL-5) plays a major role in eosinophil recruitment and function. Using an ELISA, we investigated the levels of IL-5 in the sera and blister fluid of BP patients with active disease and those in prolonged clinical remission treated with intravenous immunoglobulin, and compared it to that in normal controls. Significantly increased levels of IL-5 were detected in the serum and particularly the blister fluid of patients with active disease (P=0.0043) when compared to levels in normal controls. There was no significant difference in IL-5 levels in patients in prolonged clinical remission compared to normal control serum. In an immunoblot assay, using bovine gingival lysate as substrate, we determined the presence of IgG and IgE autoantibodies specific to basement membrane zone proteins in blister fluid and serum of BP patients. IgG autoantibodies to BPAg1 and BPAg2 were detected in both blister fluid and serum of patients, whereas IgE autoantibodies, in much lower titers, were detected to only BPAg1. Elevated levels of blood and tissue eosinophilia were observed in these patients. Based on these data, we present a preliminary hypothesis for the initiation, progression and localization of blister formation in BP.
大疱性类天疱疮(BP)是一种自身免疫性水疱性皮肤病,其特征是存在针对半桥粒正常成分(BPAg1和BPAg2)的自身抗体。皮肤病变的组织学表现为表皮下水疱和主要为嗜酸性粒细胞的真皮细胞浸润。白细胞介素5(IL-5)在嗜酸性粒细胞的募集和功能中起主要作用。我们使用酶联免疫吸附测定(ELISA)法,研究了患有活动性疾病的BP患者以及接受静脉注射免疫球蛋白治疗且处于长期临床缓解期的患者血清和疱液中IL-5的水平,并将其与正常对照组进行比较。与正常对照组相比,活动性疾病患者的血清尤其是疱液中IL-5水平显著升高(P = 0.0043)。与正常对照血清相比,处于长期临床缓解期的患者IL-5水平无显著差异。在免疫印迹分析中,我们以牛牙龈裂解物为底物,测定了BP患者疱液和血清中针对基底膜区蛋白的IgG和IgE自身抗体的存在情况。在患者的疱液和血清中均检测到了针对BPAg1和BPAg2的IgG自身抗体,而仅在低得多的滴度下检测到针对BPAg1的IgE自身抗体。在这些患者中观察到血液和组织嗜酸性粒细胞增多。基于这些数据,我们提出了一个关于BP水疱形成起始、进展和定位的初步假说。