文献简介

出版社:Indian Journal of Dermatology

作  者:Amrinder J Kanwar, Keshavamurthy Vina

编  号:

关键字:Dapsone, desmoglein, granular C3 dermatosis, IgA pemphigus, IgG/IgA pemphigus, pemphigus herpetiformis

年  份:2014   点击量:446

文献摘要


IgG/IgA pemphigus is an extremely rare subset of pemphigus, showing anti-keratinocyte cell surface antibodies of both IgG and IgA classes. Herein, we describe a unique case of IgG/IgA pemphigus with clinical features of edematous erythema and peripheral vesiculopustules. Histopathology showed the presence of subcorneal pustules and acantholytic blisters in the mid-epidermis with neutrophilic infiltration and eosinophilic spongiosis. Direct immunofluorescence of perilesional skin showed both IgG and IgA deposits to keratinocyte cell surfaces and unusual granular deposits of IgG, IgM, and C3 along basement membrane zone. On enzyme linked immunosorbent assay , the auto-antibodies were found to be reactive to desmoglein 1 antigen. Various clinical, histopathological, and immunological findings in our case overlapped with the features of IgA pemphigus, pemphigus herpetiformis, and pemphigus foliaceus. These  findings indicate that IgG/IgA pemphigus may be a transitional form between IgA pemphigus and pemphigus herpetiformis, and thus provides insight into the pathogenicity of this rare disorder.

IgG/ IgA型天疱疮是天疱疮疾病中极为罕见的一个子集,它显示IgGIgA类天疮疱两者的抗角朊细胞表面抗体。在这里,我们描述一个具有水肿性红斑及周边脓疱的临床表现的IgG/ IgA天疱疮的独特病例。组织病理显示伴有中性粒细胞浸润和嗜酸性海绵样水肿存在的角质层下脓疱和中表皮的棘层松解水疱。病灶周围皮肤的直接免疫荧光显示到角质细胞表面的IgGIgA沉积和沿基底膜带的IgGIgM抗体和C3的异常颗粒状沉积。酶联免疫吸附测定,该自身抗体被认为是反应性桥粒芯蛋白1抗原。在我们的病例中各种临床,组织病理学,免疫学和研究结果与IgA天疱疮,疱疹样天疱疮和天疱疮的特征重叠。这些结果表明,IgG/ IgA天疱疮可能是IgA天疱疮和疱疹样天疱疮之间的一种过渡形式,从而洞察到这一罕见疾病的致病性。