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Treatment of Bullous Systemic Lupus Erythematosus

机译:大疱性系统性红斑狼疮的治疗

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Bullous systemic lupus erythematosus (BSLE) is an autoantibody-mediated vesiculobullous disease in patients with SLE. Autoimmunity in BSLE is characterized by the presence of circulating anti-type VII collagen antibodies. BSLE patients often present with multiple, tense, clear fluid-filled vesicles and bullae overlying erythematous edematous plaques. Skin biopsy from BSLE patients shows subepidermal bullae with numerous neutrophils and only occasional eosinophils. Furthermore, immunofluorescence examination showed linear deposition of lgG, lgA, C3, and C1q along the basement membrane zone. BSLE patients with corticosteroids treatment constantly do not receive a marked improvement, while dapsone generally dramatically improved the skin condition. Recently, it has been reported that quite a few cases of BSLE were successfully treated with other immune suppressive drugs. Therefore, a comprehensive review of the treatment of BSLE would be beneficial to cure the disease.
机译:大疱性系统性红斑狼疮(BSLE)是SLE患者自身抗体介导的囊泡性疾病。 BSLE中的自身免疫的特征是存在循环中的抗VII型胶原蛋白抗体。 BSLE患者通常表现为多个,紧张,透明的充满液体的囊泡和覆在红斑性水肿斑块上的大疱。 BSLE患者的皮肤活检显示表皮下大疱,中性粒细胞众多,偶有嗜酸性粒细胞。此外,免疫荧光检查显示IgG,IgG,C3和C1q沿着基底膜区域线性沉积。接受皮质类固醇治疗的BSLE患者不能持续获得明显改善,而氨苯砜通常可以显着改善皮肤状况。最近,据报道,相当多的BSLE病例已成功用其他免疫抑制药物治疗。因此,全面回顾BSLE的治疗将有助于治愈该病。

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