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Clinical Relevance of Autoantibodies in Patients with Autoimmune Bullous Dermatosis

机译：自身免疫性大疱性皮肤病患者自身抗体的临床意义

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The authors present their experience related to the diagnosis, treatment, and followup of 431 patients with bullous pemphigoid, 14 patients with juvenile bullous pemphigoid, and 273 patients with pemphigus. The detection of autoantibodies plays an outstanding role in the diagnosis and differential diagnosis. Paraneoplastic pemphigoid is suggested to be a distinct entity from the group of bullous pemphigoid in view of the linear C3 deposits along the basement membrane of the perilesional skin and the “ladder” configuration of autoantibodies demonstrated by western blot analysis. It is proposed that IgA pemphigoid should be differentiated from the linear IgA dermatoses. Immunosuppressive therapy is recommended in which the maintenance dose of corticosteroid is administered every second day, thereby reducing the side effects of the corticosteroids. Following the detection of IgA antibodies (IgA pemphigoid, linear IgA bullous dermatosis, and IgA pemphigus), diamino diphenyl sulfone (dapsone) therapy is preferred alone or in combination. The clinical relevance of autoantibodies in patients with autoimmune bullous dermatosis is stressed.
机译：作者介绍了431例大疱性天疱疮，14例青少年大疱性天疱疮和273例天疱疮的诊断，治疗和随访的经验。自身抗体的检测在诊断和鉴别诊断中起着重要作用。考虑到沿病灶周围皮肤基底膜的线性C3沉积和通过免疫印迹分析证实的自身抗体的“阶梯”构型，副肿瘤性天疱疮与大疱性天疱疮是不同的。建议将IgA天疱疮与线性IgA皮肤病区分开。建议使用免疫抑制疗法，其中每两天给予一次皮质类固醇维持剂量，从而减少皮质类固醇的副作用。在检测到IgA抗体（IgA类天疱疮，线性IgA大疱性皮肤病和IgA天疱疮）之后，优选单独或组合使用二氨基二苯砜（氨苯砜）治疗。强调自身抗体在自身免疫性大疱性皮肤病患者中的临床意义。

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