Lessey Elizabeth, Li Ning, Diaz Luis, Liu Zhi
Department of Dermatology, University of North Carolina, Chapel Hill, NC 27599, USA.
Immunol Res. 2008;41(3):223-32. doi: 10.1007/s12026-008-8028-y.
Cutaneous autoimmune blistering diseases are associated with tissue injury and fluid accumulation within the skin. The initial trigger for the organ-specific damage is autoantibodies targeting skin autoantigens, which are involved in cell-cell or cell-matrix adhesion in the skin. Pemphigus autoantibodies bind to desmosomal antigens and cause intraepidermal blisters, while pemphigoid autoantibodies interact with hemidesmosomal or hemidesmosome-associated antigens and lead to dermal-epidermal junction separation. Local complement activation is a common feature for these skin blistering diseases and some complement components are readily detected in the lesional skin and blister fluids. This review summarizes the current knowledge on the role the complement system in skin blister formation. Characterization of the pathogenically relevant complement cascade and relative contribution of different pathways into complement activation provides new insights of disease pathology and may help develop better therapeutic strategies for these potentially fatal cutaneous blistering disorders.
皮肤自身免疫性水疱病与皮肤组织损伤和液体蓄积有关。器官特异性损伤的初始触发因素是针对皮肤自身抗原的自身抗体，这些自身抗原参与皮肤中的细胞 - 细胞或细胞 - 基质黏附。天疱疮自身抗体与桥粒抗原结合并导致表皮内水疱形成，而类天疱疮自身抗体与半桥粒或半桥粒相关抗原相互作用并导致真皮 - 表皮连接分离。局部补体激活是这些皮肤水疱病的共同特征，并且在皮损皮肤和水疱液中很容易检测到一些补体成分。本综述总结了关于补体系统在皮肤水疱形成中作用的当前知识。对致病相关补体级联的表征以及不同途径对补体激活的相对贡献为疾病病理学提供了新的见解，并可能有助于为这些潜在致命的皮肤水疱病制定更好的治疗策略。
