ANTIEPILIGRIN CICATRICIAL PEMPHIGOID - A SUBEPITHELIAL BULLOUS DISORDER
ANTIEPILIGRIN CICATRICIAL PEMPHIGOID - A SUBEPITHELIAL BULLOUS DISORDER
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DOI：
10.1001/archderm.130.12.1521
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发表时间：
1994-12-01
影响因子：
--
通讯作者：
YANCEY, KB
中科院分区：
文献类型：
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作者：
DOMLOGEHULTSCH, N;ANHALT, GJ;YANCEY, KB
工作背景：表肽蛋白是由培养的人角质形成细胞沉积在细胞外基质中的糖蛋白复合物，其充当这些细胞的主要整合素配体。在人类皮肤中，epiligrin存在于表皮基底膜中的透明层和致密层的界面处，据信它与锚定丝相关，并在角质形成细胞粘附中起重要作用。方法和结果：我们已经确定了6例粘膜和皮肤上皮下大疱性疾病的患者，从人角质形成细胞提取物和培养基中免疫沉淀epiligrin的基底膜自身抗体。这些患者的IgG自身抗体也结合epiligrin在人角质形成细胞的细胞外基质和表皮基底膜，如通过免疫荧光和免疫电子显微镜测定。10例临床上无法与抗癫痫自身抗体受试者区分的患者的研究（即瘢痕性类天疱疮患者）发现，虽然有7人有抗基底膜自身抗体，但后者仅针对不含epiligrin的表皮基底膜区域，（即小于或等于1：10），不与角质细胞细胞外基质反应，并且在人角质形成细胞提取物或培养基的免疫沉淀研究中不结合epiligrin（或任何其它特异性抗原）。抗epiligrin自身抗体也没有检测到在36个额外的大疱性疾病患者或6个正常volunteer.Conclusions：瘢痕性类天疱疮的研究是一种疾病的表型，其中患者的自身抗体可能针对不同成分的表皮基底膜。抗癫痫自身抗体是一组患者的特异性免疫学标志物，我们建议将其命名为抗癫痫瘢痕性类天疱疮。
Background: Epiligrin is a glycoprotein complex deposited in extracellular matrix by cultured human keratinocytes that serves as the major integrin ligand of these cells. In human skin, epiligrin is found at the interface of the lamina lucida and lamina densa in epidermal basement membrane where it is believed to be associated with anchoring filaments and plays an important role in keratinocyte adhesion.Methods and Results: We have identified six patients with a subepithelial bullous disorder of mucous membranes and skin who have IgG anti-basement membrane autoantibodies that immunoprecipitate epiligrin from human keratinocyte extracts and culture media. These patients' IgG autoantibodies also bind epiligrin in human keratinocyte extracellular matrix and epidermal basement membrane as determined by immunofluorescence and immunoelectron microscopy. Studies of 10 patients who are clinically indistinguishable from subjects with antiepiligrin autoantibodies (ie, cicatricial pemphigoid patients) found that while seven had anti-basement membrane autoantibodies, the latter are directed exclusively against a region of epidermal basement membrane that does not contain epiligrin, are present in low titer (ie, less than or equal to 1:10), do not react with keratinocyte extracellular matrix, and do not bind epiligrin (or any other specific antigen) in immunoprecipitation studies of human keratinocyte extracts or media. Antiepiligrin autoantibodies were also not detected in studies of 36 additional patients with bullous diseases or six normal volunteers.Conclusions: Cicatricial pemphigoid is a disease phenotype in which patients' autoantibodies may target different constituents of epidermal basement membrane. Antiepiligrin autoantibodies are a specific immunologic marker for a group of patients with a disease entity that we propose to designate antiepiligrin cicatricial pemphigoid.