Department of Dermatology, University of Lübeck, Lübeck, Germany.
Exp Dermatol. 2010 May;19(5):458-63. doi: 10.1111/j.1600-0625.2010.01069.x. Epub 2010 Feb 16.
Pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are intraepidermal blistering skin diseases. PV is characterised by autoantibodies directed against desmoglein (Dsg) 3 and in patients with the mucocutaneous variant also against Dsg 1, whereas in PF, only Dsg 1 is targeted. Here, ectodomains of Dsg 3 and Dsg 1 were recombinantly expressed in a human cell line (HEK293) and applied as authentic solid phases in ELISA test systems. Autoantibodies against Dsg 3 and/or Dsg 1 could be detected in 71 (100%) of 71 PV sera and against Dsg 1 in 48 (96%) of 50 PF sera. Control sera showed reactivity with Dsg 3 and Dsg 1 in 0.2% and 0.7%, respectively, of 401 healthy blood donors and in 2.1% of 48 randomly selected patients with bullous pemphigoid. No reactivity with Dsg 1 and 3 was detected in 21 patients with linear IgA disease. For both pemphigus variants, a statistically significant correlation between clinical severity and autoantibody levels was observed as demonstrated for 10 PV and 5 PF patients. In conclusion, the use of the ectodomains of Dsg 3 and 1 as target antigens expressed in a human cell line resulted in sensitive and specific ELISA systems for both diagnosis and monitoring of PV and PF.
寻常型天疱疮（PV）和落叶型天疱疮（PF）是表皮内水疱性皮肤病。PV 的特征是自身抗体针对桥粒芯糖蛋白 3（Dsg）3，黏膜皮肤型患者也针对 Dsg 1，而 PF 仅针对 Dsg 1。在此，我们在人细胞系（HEK293）中重组表达了 Dsg 3 和 Dsg 1 的胞外结构域，并将其作为 ELISA 测试系统中的真实固相进行应用。在 71 例（100%）PV 血清中检测到针对 Dsg 3 和/或 Dsg 1 的自身抗体，在 50 例 PF 血清中检测到针对 Dsg 1 的自身抗体。对照血清在 401 名健康献血者中分别有 0.2%和 0.7%的反应性，在 48 名随机选择的大疱性类天疱疮患者中分别有 2.1%的反应性。在 21 例线性 IgA 疾病患者中未检测到针对 Dsg 1 和 3 的反应性。对于这两种天疱疮变体，我们观察到临床严重程度与自身抗体水平之间存在统计学显著相关性，这在 10 例 PV 和 5 例 PF 患者中得到了证实。总之，使用在人细胞系中表达的 Dsg 3 和 1 的胞外结构域作为靶抗原，可产生用于诊断和监测 PV 和 PF 的敏感和特异性 ELISA 系统。
