Hashimoto T
Department of Dermatology, School of Medicine, Keio University, Tokyo, Japan.
Keio J Med. 1995 Dec;44(4):115-23. doi: 10.2302/kjm.44.115.
Autoimmune blistering skin diseases develop separation either between epidermal keratinocytes or dermo-epidermal junction. Recent studies have revealed that the autoantigens for these diseases are components of either the desmosome, the cell adhesion junction between keratinocytes, or the hemidesmosome complex, cell adhesion machinery at the dermo-epidermal junction. Thus, the major pemphigus antigens are desmogleins, one of desmosomal cadherins. Both the 230 kD and 180 kD bullous pemphigoid antigens are present in the hemidesmosome, and epidermal basement membrane zone-specific extracellular matrices, epiligrin and type VII collagen, are detected by sera of cicatricial pemphigoid and epidermolysis bullosa acquisita, respectively. Furthermore, animal model studies using newborn mice have revealed that these autoantibodies are really pathogenic and can induce blister formation by passive transfer into mice. Therefore, these skin diseases seem to be a typical model for various autoimmune diseases, for most of which the role of autoantibodies has not yet been revealed.
自身免疫性水疱性皮肤病在表皮角质形成细胞之间或真皮 - 表皮交界处发生分离。最近的研究表明，这些疾病的自身抗原是桥粒（角质形成细胞之间的细胞粘附连接）或半桥粒复合体（真皮 - 表皮交界处的细胞粘附机制）的组成部分。因此，天疱疮的主要抗原是桥粒芯糖蛋白，它是桥粒钙粘蛋白之一。230kD和180kD大疱性类天疱疮抗原都存在于半桥粒中，瘢痕性类天疱疮和获得性大疱性表皮松解症的血清分别检测到表皮基底膜带特异性细胞外基质表皮整联配体蛋白和VII型胶原。此外，使用新生小鼠的动物模型研究表明，这些自身抗体确实具有致病性，并且可以通过被动转移到小鼠体内诱导水疱形成。因此，这些皮肤病似乎是各种自身免疫性疾病的典型模型，其中大多数疾病中自身抗体的作用尚未明确。
