大疱性类天疱疮免疫发病机制的研究进展

Advances in the immunologic pathogenesis of bullous pemphigoid

大疱性类天疱疮作为常见的自身免疫性疱病，国内外学者一直进行其发病机制的研究。目前认为，IgG是犬疱件类天疱疮患者血清中主要的致病性自身抗体类型，与BP180等自身抗原结合后可固定并激活补体，导致肥大细胞脱颗粒，激活浸润的炎症细胞并使其释放蛋白水解酶，引起真表皮连接部位的重要蛋白结构受损，最终导致表皮下水疱的发生。研究表明，体液免疫和细胞免疫均参与大疱性类大疱疮的发病过程，但久疱性类天疱疮自身抗体及可能存在的自身抗原的来源及嗜酸粒细胞仵发病过程中的作用尚水完全清楚。

Bullons pemphigoid （BP） is a common autoimmune blistering skin disease. Studies on its inunuoologic pathogeuesis have been always carried out at home and abroad. IgG autoantibodies, which are the predominant type of pathogenic autoantibodies, can bind to muhiple BP180 epitopes, result in the fixatinn and activation of complement, degrauulation of resident mast cells, activation of infiltrating inflammatory cells and release of proteinases followed by the loss of cell-matrix adhesion structure at the dermal-epidermal junction, and finally cause the formation of subepidermal blisters. Although studies have shown that both humoral and cellular immunity are involved, little is known about the resource of autoantibodies, probalble existent autoantigens, and the roles of eosinophils in the pathogenesis of BP.