天疱疮患者表皮中桥粒芯蛋白1的分布模式

The distribution pattern of desmoglein 1 in the epidermis of patients with pemphigus

目的:探讨桥粒芯蛋白1在天疱疮患者表皮中的分布模式。方法:用ELISA方法测定患者血清中的桥粒芯蛋白抗体滴度,同时用免疫组化的方法测定天疱疮患者皮肤中桥粒芯蛋白1的分布情况,并与其他表皮内发生棘刺松解的疾病以及正常人对照。结果:在20例特发性天疱疮中12例患者血清同时存在桥粒芯蛋白1和桥粒芯蛋白3的抗体,桥粒芯蛋白1在这12例患者皮肤中均呈现异常块状模式。8例患者的血清中只有桥粒芯蛋白1的抗体,其中4例患者表皮中桥粒芯蛋白1的分布为异常块状模式,4例为正常网状模式。6例药物诱发天疱疮中有1例患者血清中同时存在桥粒芯蛋白1和桥粒芯蛋白3的抗体,5例患者的血清中只有桥粒芯蛋白1的抗体,桥粒芯蛋白1在药物诱发天疱疮表皮中的分布模式为4例表现为正常网状模式,2例为异常的块状模式。结论:桥粒芯蛋白1在天疱疮患者表皮中的分布模式可出现正常的网状模式,但在血清中同时存在桥粒芯蛋白1和3抗体的患者中均呈现异常的块状模式。

Objective： To investigate the distribution pattern of desmoglein 1 in the epidermis of patients with pemphigus. Methods： The ELISA assay was used to detect the titers of the antibodies against desmogleins in the serum of patients with idiopathic pemphigus, the immunohistochemistry assay was used to detect the distribution pattern of desmoglein 1 in the epidermis of these patients. These results were compared with those in the drug-induced pemphigus and the heahhy controls. Results： Twenty cases of idiopathic pemphigus and 6 cases of drug-induced pemphigus were studied. In 12 out of 20 patients with idiopathic pemphigus, both antibodies against desmoglein 1 and desmoglein 3 were detected in the serum, and an abnormal patchy distribution of desmoglein 1 was shown in the skin lesions. In other 8 patients with idiopathic pemphigus, only the antibody against desmoglein 1 was found in the serum. Meanwhile, an abnormal patchy distribution of desmoglein 1 in the skin specimens was found in 4 of these 8 patients, and a normal retiform distribution of desmoglein 1 was shown in other 4 patients. In 6 patients with drug-induced pemphigus, serum antibodies against both desmoglein 1 and desmoglein 3 were detected in one patient, and only desmoglein 1 antibody was found in the rest of 5 patients. An abnormal patchy distribution of desmoglein 1 in the epidermis was revealed in 2 of the 6 patients, and a normal retiform distribution of it was revealed in other 4 patients. Conclusion： A normal retiform distribution pattern of desmoglein 1 is seen in the epidermis of patients with pemphigus, and an abnormal patchy distribution pattern desmoglein 1 could he seen when both anti-desmoglein 1 antibody and anti-desmoglein 3 antibody are detected in the serum of the patients.