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大疱性类天疱疮相关性肾病:两例报告及文献复习

Bullous pemphigoid-associated nephropathy: report of two cases and review of the literature.

作者信息

Ross E A, Ahmed A R

机构信息

Nephrology Section, University of California, Irvine-Long Beach Medical Program 90822.

出版信息

Am J Kidney Dis. 1989 Sep;14(3):225-9. doi: 10.1016/s0272-6386(89)80076-9.

DOI:10.1016/s0272-6386(89)80076-9
PMID:2528289
Abstract

Bullous pemphigoid has previously been reported in association with a variety of renal lesions. Two additional cases are presented in this report in which the nephropathy preceded the onset of the skin disease: one case with membranous glomerulopathy and one case of renal allograft rejection with concurrent membranous pathology. Both patients had positive immunofluorescence of the skin, typical of bullous pemphigoid. Institution of systemic corticosteroid therapy resulted in a satisfactory clinical response and cessation of the blistering process. These cases and a review of the literature suggest that the occurrence of an immune process involving these two different basement membranes is not merely coincidental. Many cases have been described in which the severity of the skin lesions paralleled that of the renal disease. Although the possibility of multiple distinct autoimmune processes cannot be excluded, anti-basement-zone antibody interactions or allograft rejection-induced immune stimulation are possible unifying mechanisms for the simultaneous skin and renal involvement observed in these two cases.

摘要

此前已有大疱性类天疱疮与多种肾脏病变相关的报道。本报告又介绍了另外两例肾病先于皮肤病发作的病例:一例为膜性肾小球病,另一例为肾移植排斥反应并发膜性病变。两名患者皮肤免疫荧光均呈阳性,为典型的大疱性类天疱疮。全身使用皮质类固醇激素治疗取得了满意的临床疗效,水疱形成过程停止。这些病例以及文献回顾表明,涉及这两种不同基底膜的免疫过程的发生并非仅仅是巧合。已有许多病例描述皮肤病变的严重程度与肾脏疾病的严重程度相当。尽管不能排除存在多种不同自身免疫过程的可能性,但抗基底膜区抗体相互作用或移植排斥诱导的免疫刺激可能是这两例中同时出现皮肤和肾脏受累的统一机制。

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