抗p200类天疱疮7例临床及免疫血清学特征分析

Clinical and immunoserological features of 7 cases of anti-p200 pemphigoid

目的总结抗p200类天疱疮患者的临床和免疫血清学特征。方法收集2015年1月至2021年10月在中国医学科学院皮肤病医院确诊为抗p200类天疱疮的患者资料,回顾性分析其临床和免疫血清学特征。结果纳入7例抗p200类天疱疮患者,盐裂皮肤-间接免疫荧光实验显示,7例患者血清IgG抗体均结合于盐裂皮肤的真皮侧,以真皮提取物为底物的免疫印迹显示,在相对分子质量200000蛋白处有条带。4例呈经典型大疱性类天疱疮样损害,2例初起呈湿疹样损害,1例类似线状IgA大疱性皮病。6例循环IgG抗体可识别层粘连蛋白γ1-C端重组蛋白。4例患者接受不同剂量系统糖皮质激素治疗,其中1例对高剂量系统糖皮质激素(相当于泼尼松1.4 mg·kg^(-1)·d^(-1))治疗抵抗;2例对米诺环素、氨苯砜治疗反应好;1例失访。4例患者在随访平均22.5个月时达到完全缓解停药;2例在随访平均8个月时达到最小剂量治疗保持完全缓解。结论抗p200类天疱疮临床表现多样,重组Lnγ1-C端可作为可靠的抗原底物用于检测抗p200类天疱疮患者自身抗体;部分患者最终可达到完全缓解并停药。

Objective To analyze clinical and immunoserological features of patients with anti-p200 pemphigoid.Methods Clinical data were collected from patients with confirmed anti-p200 pemphigoid in Hospital of Dermatology,Chinese Academy of Medical Sciences from January 2015 to October 2021,and their clinical and immunoserological characteristics were retrospectively analyzed.Results Seven patients with anti-p200 pemphigoid were included.Indirect immunofluorescence on salt-split skin(IIF-SSS)showed that serum IgG antibodies of the 7 patients were located in the dermis of the salt-split skin,and Western blot analysis with dermal extracts as substrates revealed a protein band with a relative molecular mass of 200000.Four patients presented with classic bullous pemphigoid-like skin lesions,2 initially presented with eczematous lesions,and 1 presented with linear IgA bullous dermatosis-like skin lesions.Circulating IgG antibodies could recognize the recombinant lamininγ1 C-terminal region in 6 cases.Four patients received different doses of systemic glucocorticoids,1 of whom was resistant to high-dose systemic glucocorticoids(equivalent to 1.4 mg·kg^(-1)·d^(-1) prednisone);2 responded well to minocycline and dapsone;1 was lost to follow-up.Four patients achieved complete remission and discontinued the treatment at a mean follow-up of 22.5 months;2 received complete remissiona on minimal therapy at a mean follow-up of 8 months.Conclusion Patients with anti-p200 pemphigoid presented with heterogeneous clinical manifestations,and the recombinant C-terminal fragment of lamininγ1 can serve as a reliable antigen substrate for the detection of autoantibodies in patients with anti-p200 pemphigoid;some patients can eventually achieve complete remission off treatment.