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首页> 外文期刊>Clinics in dermatology >Life-threatening bullous dermatoses: Pemphigus vulgaris.
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Life-threatening bullous dermatoses: Pemphigus vulgaris.

机译:威胁生命的大疱性皮肤病:寻常型天疱疮。

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摘要

Pemphigus vulgaris (PV) is a rare autoimmune bullous dermatosis with a high mortality rate if untreated. The disease results from autoimmunity to normal components of keratinocyte cell membrane (desmogleins 3 and 1) belonging to the cadherin supergene family. Standard therapy for PV is based on a combined administration of high-dosed glucocorticoids and immunosuppressive drugs. In patients with severe, life-threatening, or recalcitrant PV, stronger therapeutic options should be considered, such as 'pulse-therapy' with discontinuous intravenous infusion of megadoses of immunosuppressive drugs over a short-time, plasmapheresis, and extracorporeal immunoadsorption of pathogenic autoantibodies using the extracellular domain of the PV main antigen (desmoglein 3) produced by baculovirus or, more recently, a tryptophan-linked polyvinyl alcohol adsorber.
机译:寻常型天疱疮(PV)是一种罕见的自身免疫性大疱性皮肤病,如果不治疗,其死亡率很高。该疾病是由于对属于钙粘着蛋白超基因家族的角质形成细胞膜(桥粒蛋白3和1)的正常成分进行自身免疫所致。 PV的标准疗法基于高剂量糖皮质激素和免疫抑制剂的联合给药。对于严重,危及生命或顽固性PV的患者,应考虑采用更强的治疗选择,例如短期内不连续静脉内注入大量免疫抑制药物,血浆置换术以及病原体自身抗体的体外免疫吸附的“脉冲疗法”使用杆状病毒或更近的色氨酸连接的聚乙烯醇吸附剂产生的PV主要抗原(桥粒芯蛋白3)的胞外域。

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