文献简介

出版社:American Medical Association

作  者:Imke Düker, MD; Jörg Schaller, MD

编  号:

关键字:IgA Pemphigus

年  份:2009   点击量:435

文献摘要

Background: IgA pemphigus is a rare neutrophilic acantholytic autoimmune disease that is characterized by IgA deposits on keratinocyte cell surfaces. Clinically and histologically, IgA pemphigus is divided into 2 major subtypes: subcorneal pustular dermatosis and intraepidermal neutrophilic IgA dermatosis. We report the first case of subcorneal pustular dermatosistype IgA pemphigus that showed reactivity to all 3 isoforms of the desmocollin family by indirect immunofluorescence microscopy of COS7 cells transfected with desmocollin 1, 2, or 3.
研究背景:IgA天疱疮是一种罕见的嗜中性粒细胞棘层松解性自身免疫疾病,它的特征为角质形成细胞的细胞表面的IgA沉积。根据临床和组织学,IgA型天疱疮被分为2个主要亚型:角层下脓疱性皮病型和表皮内嗜中性皮病型。我们报道角层下脓疱性皮病型IgA型天疱疮的首个病例,该病例通过子转染桥粒胶蛋白12,或3COS7细胞的间接免疫荧光显微镜 ,表现了桥粒胶蛋白家族的所有3个亚型的反应。

 

Observations: We describe a 94-year-old woman with IgA pemphigus with a unique immunopathologic pattern. Direct immunofluorescence microscopy revealed IgA deposits throughout the entire epidermis, with stronger staining in the upper epidermis. The autoantibodies from this patient did not show IgA or IgG reactivity with desmogleins via immunoblotting or enzyme-linked immunosorbent assay. By indirect immunofluorescence by the use of COS7 cells transfected with desmocollin 1, 2, or 3, IgA autoantibodies in a serum sample from our patient clearly reacted with all of them.

观察:我们描述一个具有独特的免疫病理模式的IgA型天疱疮的94岁妇女。直接免疫荧光显微镜显示整个表皮的IgA沉积,上表皮强染色。通过免疫印迹或酶联免疫吸附试验,从该患者的自身抗体没有通过免疫印迹或酶联免疫吸附测定法显示桥粒芯蛋白中IgA的或IgG的反应性。对转染桥粒胶蛋白12、或3COS7细胞使用间接免疫荧光法后,我们患者的血清样本中的IgA抗体与他们都有明显地反应。


Conclusions: The pathophysiology and autoantigen profile of bullous autoimmune diseases, especially pemphigus and its subforms, are more complex than previously believed. Because pemphigus seems to be a heterogeneous disorder, further studies are needed to evaluate the complexity of the disease.

研究结论:大疱性自身免疫性疾病,特别是天疱疮和它的子集的病理生理学及自身抗原简况比我们原先认为的更复杂。因为天疱疮似乎是一个异质性疾病,我们还需要进一步研究,以评估疾病的复杂性。