本文综述遗传性补体缺乏及其有关疾病的近期知识,特别着重于皮肤病学有关的方面.补体是由一系列不同的蛋白质构成的效应系统,其中大多数蛋白质具有酶的活性.在正常情况下以生物学非活性的形式存在,通过一系列级联式高特异性生化反应而激活,此种反应由数种特异性抑制蛋白质调节,目前已知的补体成分不下15种. This review summarizes the recent lack of knowledge about inherited complement deficiency and its related illnesses, with particular emphasis on dermatology-related aspects. Complement is an effector system consisting of a series of different proteins, most of which have enzymatic activity. Under normal circumstances It exists in a biologically inactive form and is activated by a series of cascade high-specific biochemical reactions that are regulated by several specific inhibitory proteins. There are currently 15 known complement components.