Binding of autoantibodies is not restricted to desmosomes in pemphigus vulgaris: Comparison of 14 cases of pemphigus vulgaris and 10 cases of pemphigus foliaceus studied by Western immunoblot and immunoelectron microscopy
Binding of autoantibodies is not restricted to desmosomes in pemphigus vulgaris: Comparison of 14 cases of pemphigus vulgaris and 10 cases of pemphigus foliaceus studied by Western immunoblot and immunoelectron microscopy
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DOI：
10.1007/bf02507101
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发表时间：
1996-06-01
影响因子：
3
通讯作者：
Dubertret, L
中科院分区：
文献类型：
--
作者：
Bedane, C;Prost, C;Dubertret, L
寻常型天疱疮（PV）和落叶型天疱疮（PF）是一种自身免疫性水疱性疾病，其特征是细胞间粘附功能丧失和抗表皮钙粘蛋白的自身抗体。PF抗原被确定为桥粒芯糖蛋白I，严格定位于桥粒上，而PV抗原的超微结构定位至今仍不清楚，存在争议。为了进一步研究这个问题，我们比较了14例PV和10例PF患者的免疫沉积物的直接和间接免疫电镜（IEM）的位置。入选标准基于临床特征、组织学水平的切割和通过Western印迹对上皮牛舌提取物的循环抗体的表征，IEM在未固定的0.7 mm皮肤切片上进行直接技术或在正常皮肤上进行间接技术，使用过氧化物酶标记。在PF中，通过直接和间接IEM，免疫沉积物位于桥粒的细胞外部分（桥粒胶）在所有研究的样品中，在PV中，通过直接和间接IEM，沉积物位于桥粒胶和沿着角质形成细胞膜的大部分没有桥粒结构的18个研究的样品中的15个和3个样品中的仅在桥粒胶上。这些结果表明，与PF相反，PV中的靶抗原并不总是局限于桥粒。由于已报道各种类型的粘附连接介导表皮中的细胞粘附，PV抗原可能是桥粒和其他粘着斑的组分。
Pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are autoimmune blistering diseases characterized by a Loss of cell-cell adhesion and by autoantibodies directed against epidermal cadherins, PF antigen has been established as desmoglein I which is located strictly on the desmosome, whereas the precise ultrastructural localization of PV antigen remains unclear and controversial to date. To further investigate this question, we compared the location of immune deposits in 14 patients with PV and 10 patients with PF by both direct and indirect immunoelectron microscopy (IEM). Inclusion criteria were based upon clinical features, histological level of cleavage and characterization of circulating antibodies by Western blot on epithelial bovine tongue extracts, IEM was performed on unfixed 0.7-mm slices of skin for the direct technique or on normal skin for the indirect technique using peroxidase labelling. In PF, by both direct and indirect IEM, immune deposits were located on the extracellular part of desmosomes (desmoglea) in all the samples studied, In PV, by both direct and indirect IEM, deposits were situated on the desmoglea and along large portions of the keratinocyte membrane without desmosomal structures in 15 of the 18 samples studied and only on the desmoglea in 3 samples. These results suggest that, in contrast to PF, the target antigen in PV is not always restricted to desmosomes. As various types of adherens junctions have been reported to mediate cell adhesion in the epidermis, the PV antigen could be a component of desmosomes and of other focal adhesions.