Huang Cheng-Hung, Chen Chih-Chiang, Wang Chi-Jui, Chang Yun-Ting, Liu Han-Nan
Department of Dermatology, Taipei Veterans General Hospital, and National Yang-Ming University School of Medicine, Taipei, Taiwan, ROC.
J Chin Med Assoc. 2007 Feb;70(2):65-70. doi: 10.1016/S1726-4901(09)70304-1.
Pemphigus is an acquired autoimmune intraepidermal blistering disease that is divided into 2 major subtypes: pemphigus vulgaris (PV) and pemphigus foliaceus (PF). Patients with pemphigus have circulating anti-desmoglein (Dsg)1 and/or anti-Dsg3 IgG autoantibodies. Recently, a novel commercial enzyme-linked immunosorbent assay (ELISA) against Dsg1 and Dsg3 has been established and found to be extremely sensitive and specific. To date, the usefulness of Dsg1 and Dsg3 ELISA in the diagnosis of pemphigus in the Taiwanese population has never been reported.
Serum samples were obtained from 143 patients, including 20 patients with PV, 9 patients with PF, 72 patients with bullous pemphigoid, 1 patient with dermatitis herpetiformis and 41 patients with non-autoimmune blistering diseases. They were tested for anti-Dsg1 and anti-Dsg3 reactivity by ELISA.
Seventeen of 20 PV sera (85%) exceeded the cut-off value of Dsg3 ELISA, and 9 of 9 PF sera (100%) exceeded the cut-off value of Dsg1 ELISA, while only 1 (0.88%) and 3 (2.6%) of 114 non-pemphigus sera exceeded the cut-off values of Dsg3 and Dsg1 ELISAs, respectively. Thus, the sensitivity and specificity of Dsg3 ELISA were 85% and 99.1%, while the sensitivity and specificity of Dsg1 ELISA were 100% and 97.4%, respectively. The correlation between ELISA scores and disease activity along the time course was examined in 6 PV patients and 1 PF patient, and the result was equivocal.
Dsg1 and Dsg3 ELISAs provide a simple, highly sensitive and specific method that can serve as a useful adjunct tool for the initial diagnosis of pemphigus.
天疱疮是一种获得性自身免疫性表皮内水疱性疾病，主要分为两大亚型：寻常型天疱疮（PV）和落叶型天疱疮（PF）。天疱疮患者体内存在循环抗桥粒芯糖蛋白（Dsg）1和/或抗Dsg3 IgG自身抗体。最近，一种针对Dsg1和Dsg3的新型商业酶联免疫吸附测定（ELISA）已被建立，并发现其具有极高的敏感性和特异性。迄今为止，Dsg1和Dsg3 ELISA在台湾人群天疱疮诊断中的应用尚未见报道。
收集了143例患者的血清样本，其中包括20例PV患者、9例PF患者、72例大疱性类天疱疮患者、1例疱疹样皮炎患者和41例非自身免疫性水疱性疾病患者。通过ELISA检测他们的抗Dsg1和抗Dsg3反应性。
20例PV血清中有17例（85%）超过Dsg3 ELISA的临界值，9例PF血清中有9例（100%）超过Dsg1 ELISA的临界值，而114例非天疱疮血清中分别只有1例（0.88%）和3例（2.6%）超过Dsg3和Dsg1 ELISA的临界值。因此，Dsg3 ELISA的敏感性和特异性分别为85%和99.1%，而Dsg1 ELISA的敏感性和特异性分别为100%和97.4%。对6例PV患者和1例PF患者在病程中ELISA评分与疾病活动度之间的相关性进行了检测，结果不明确。
Dsg1和Dsg3 ELISA提供了一种简单、高度敏感和特异的方法，可作为天疱疮初步诊断的有用辅助工具。
