Pemphigus and bullous pemphigoid are autoantibody-mediated blistering skin diseases. In pemphigus, keratinocytes in epidermis and mucous membranes lose cell-cell adhesion, and in pemphigoid, the basal keratinocytes lose adhesion to the basement membrane. Pemphigus lesions are mediated directly by the autoantibodies, whereas the autoantibodies in pemphigoid fix complement and mediate inflammation. In both diseases, the autoantigens have been cloned and characterized; pemphigus antigens are desmogleins (cell adhesion molecules in desmosomes), and pemphigoid antigens are found in hemidesmosomes (which mediate adhesion to the basement membrane). This knowledge has enabled diagnostic testing for these diseases by enzyme-linked immunosorbent assays and dissection of various pathophysiological mechanisms, including direct inhibition of cell adhesion, antibody-induced internalization of antigen, and cell signaling. Understanding these mechanisms of disease has led to rational targeted therapeutic strategies.

中文翻译：

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发病机理：天疱疮和大疱性天疱疮。

天疱疮和大疱性天疱疮是自身抗体介导的水疱性皮肤疾病。在天疱疮中，表皮和粘膜中的角质形成细胞失去细胞粘附，而在天疱疮中，基底角质形成细胞失去与基底膜的粘附。天疱疮的病变直接由自身抗体介导，而天疱疮中的自身抗体修复补体并介导炎症。在这两种疾病中，自身抗原均已被克隆和鉴定。天疱疮抗原是桥粒芯蛋白（桥粒中的细胞粘附分子），而天疱疮抗原则存在于半桥粒中（介导与基底膜的粘附）。这些知识可通过酶联免疫吸附测定和解剖各种病理生理机制（包括直接抑制细胞粘附，抗体诱导的抗原内在化和细胞信号转导。了解这些疾病的机制已导致合理的靶向治疗策略。