Ishiura Nobuko, Fujimoto Manabu, Watanabe Rei, Nakashima Hiroko, Kuwano Yoshihiro, Yazawa Norihito, Echigo Takeshi, Okochi Hitoshi, Tamaki Kunihiko
Department of Dermatology, Graduate School of Medicine, University of Tokyo, Bunkyo, Tokyo, Japan.
J Dermatol Sci. 2008 Feb;49(2):153-61. doi: 10.1016/j.jdermsci.2007.08.008. Epub 2007 Oct 24.
Bullous pemphigoid (BP) is a subepidermal blistering disease characterized by autoantibodies against the hemidesmosomal proteins, BP180 and BP230. NC16A, a non-collagenous stretch of the BP180 ectodomain is the primary target of pathogenic IgG antibodies. Whereas IgG anti-BP180 autoantibodies play a primary role in the pathogenesis, there is a growing number of data regarding the potential pathogenic roles of IgE class autoantibodies in BP.
To examine the levels of IgG and IgE autoantibodies against BP180 and BP230, and to investigate mutual association and clinical relevance.
Sera obtained from 67BP patients and 36 healthy donors were subjected to ELISA assays to measure serum IgG and IgE levels of anti-BP180 and anti-BP230 antibodies.
IgG anti-BP180 antibodies were positive in 63 (94%) of 67BP patients. IgG anti-BP230, IgE anti-BP180, and IgE anti-BP230 antibodies were found in 48 (72%), 20 (30%) and 45 (67%), respectively. IgG anti-BP180 levels were correlated with the affected areas. IgG anti-BP230 antibodies tended to increase in proportion to elongation of disease duration. IgE anti-BP230 levels showed a strong association with local eosinophil accumulation, while the levels were reversely related with the affected areas in BP.
IgE autoantibodies to BP180 and BP230 are detected at high frequencies in BP. IgE anti-BP230 antibodies may have a role in attracting eosinophils to the skin lesions.
大疱性类天疱疮(BP)是一种表皮下疱病,其特征是针对半桥粒蛋白BP180和BP230产生自身抗体。BP180胞外域的非胶原性片段NC16A是致病性IgG抗体的主要靶点。虽然IgG抗BP180自身抗体在发病机制中起主要作用,但关于IgE类自身抗体在BP中的潜在致病作用的数据越来越多。
检测抗BP180和BP230的IgG和IgE自身抗体水平,并研究它们之间的相互关系及临床相关性。
对67例BP患者和36名健康供者的血清进行ELISA检测,以测定抗BP180和抗BP230抗体的血清IgG和IgE水平。
67例BP患者中有63例(94%)IgG抗BP180抗体呈阳性。IgG抗BP230、IgE抗BP180和IgE抗BP230抗体的阳性率分别为48例(72%)、20例(30%)和45例(67%)。IgG抗BP180水平与受累面积相关。IgG抗BP230抗体倾向于随着病程延长而升高。IgE抗BP230水平与局部嗜酸性粒细胞聚集密切相关,而在BP中该水平与受累面积呈负相关。
在BP患者中可高频检测到针对BP180和BP230的IgE自身抗体。IgE抗BP230抗体可能在吸引嗜酸性粒细胞至皮肤病变部位中起作用。