Bullous and cicatricial pemphigoid.
Bullous and cicatricial pemphigoid.
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大疱性和瘢痕性类天疱疮。
DOI：
10.1016/0896-8411(91)90004-v
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发表时间：
1991
影响因子：
12.8
通讯作者：
Morrison,LH
中科院分区：
文献类型：
--
作者：
Anhalt,GJ;Morrison,LH
大疱性类天疱疮（BP）和瘢痕性类天疱疮是皮肤粘膜水疱性疾病，其特征是上覆上皮与基质分离。IgG和补体成分通过上皮的透明层沉积在所有受影响的组织中的水疱形成水平。一次抗体应答属于IgG 4亚类，这些自身抗体识别的抗原已被证明是复层鳞状上皮细胞半桥粒特有的230 kD和180 kD跨膜蛋白[1，2]。尽管怀疑这些抗原在细胞-基质粘附中是重要的，但这尚未得到证实。Stanleyet等人最近确定了230 kD抗原的C末端部分的序列[3]，Diazet等人分离了编码180 kD抗原的cDNA [4]。关于这些抗原的结构数据应该证明是定义其假定功能的关键。因此，BP被认为是一种自身免疫性疾病，其中皮肤病变可能仅仅是这些抗半桥粒自身抗体与特异性抗原结合的结果，但这一假设的确切证据并不完整[5]。
Bullous pemphigoid (BP) and cicatricial pemphigoid are blistering mucocutaneous diseases characterized by detachment of the overlying epithelium from its stroma. IgG and complement components are deposited in all affected tissue at the level of blister formation—through the lamina lucida of the epithelium. The primary antibody response is of the IgG 4 subclass, and the antigens recognized by these autoantibodies have been shown to be 230 kD and 180 kD transmembrane proteins unique to the hemidesmosome of stratified squamous epithelial cells [1,2]. Although it is suspected that these antigens are important in cell-substrate adhesion, this has not been proven. Stanleyet al.have recently defined the sequence of a portion of the C terminal end of the 230 kD antigen [3] and Diazet al.have isolated a cDNA encoding for the 180 kD antigen [4]. Structural data regarding these antigens should prove critical to definition of their presumed function. Therefore, BP is felt to be an autoimmune disease where the cutaneous lesions may solely be a consequence of binding of these antihemidesmosome autoantibodies to the specific antigen, but definitive proof of this assumption is incomplete [5].