The clinical phenotype of pemphigus is defined by the anti-desmoglein autoantibody profile
The clinical phenotype of pemphigus is defined by the anti-desmoglein autoantibody profile
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DOI：
10.1016/s0190-9622(99)70183-0
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发表时间：
1999-02-01
影响因子：
13.8
通讯作者：
Nishikawa, T
中科院分区：
文献类型：
--
作者：
Amagai, M;Tsunoda, K;Nishikawa, T
背景资料：一些寻常型天疱疮（PV）患者有粘膜糜烂伴轻微皮肤受累（粘膜优势型），其他患者除粘膜受累外还表现出广泛的皮肤水疱和糜烂（粘膜皮肤型）。落叶型天疱疮（PF）的患者显示只有皮肤involvement.Objective：本研究的目的是确定是否有粘膜显性PV，粘膜皮肤PV，PF之间的自身抗体谱的差异。方法：抗体滴度对桥粒芯糖蛋白1（Dsg1）和桥粒芯糖蛋白3（Dsg3）进行了测定与酶联免疫吸附试验使用重组Dsg1和Dsg3。血清中获得的临床活动性疾病从24例粘膜占主导地位的PV，20与粘膜皮肤PV，和23与PF。结果：所有的血清样品从患者的粘膜占主导地位的PV血清Dsg1阴性，但对Dsg3阳性。皮肤粘膜PV患者的所有血清样本均为Dsg1和Dsg3阳性。结论：天疱疮各亚型均存在不同的抗Dsg自身抗体谱，提示天疱疮的临床表型由自身抗体谱决定。
Background: Some patients with pemphigus vulgaris (PV) have mucous membrane erosions with minimal skin involvement (mucosal dominant type), and others show extensive skin blisters and erosions in addition to mucous membrane involvement (mucocutaneous type). Patients with pemphigus foliaceus (PF) show only skin involvement.Objective: The purpose of this study is to determine whether there is a difference in autoantibody profile among mucosal dominant PV, mucocutaneous PV, and PF.Methods: Antibody titer against desmoglein 1 (Dsg1) and desmoglein 3 (Dsg3) were measured with enzyme-linked immunosorbent assay using recombinant Dsg1 and Dsg3. Sera were obtained during clinically active disease from 24 patients with mucosal dominant PV, 20 with mucocutaneous PV, and 23 with PF.Results: All sera samples from patients with mucosal dominant PV sera were negative against Dsg1 but positive against Dsg3. All sera samples from those with mucocutaneous PV were positive against both Dsg1 and Dsg3. All sera samples from patients with PF were positive against Dsg1, but negative against Dsg3.Conclusion: Each subtype has its own anti-Dsg autoantibody profile, indicating that the clinical phenotype of pemphigus is defined by the autoantibody profile.