Bullous systemic lupus erythematosus as an initial manifestation of SLE
Bullous systemic lupus erythematosus as an initial manifestation of SLE
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DOI：
10.1111/j.1346-8138.2005.tb00894.x
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发表时间：
2005-12-01
影响因子：
3.1
通讯作者：
Iwatsuki, K
中科院分区：
文献类型：
--
作者：
Fujimoto, W;Hamada, T;Iwatsuki, K
大疱性系统性红斑狼疮(BSLE)是一种罕见的系统性红斑狼疮，常与对III型胶原的自身免疫有关。我们描述一位45岁的女性系统性红斑狼疮患者，以水泡性皮损为首发症状。患者首先注意到广泛的荨麻疹、红斑性皮疹，并伴有紧张的水泡、糜烂和结壳。她被诊断为大疱性类天疱疮，并接受了倍他米松一个月的疗程。由于出现明显的蛋白尿，随后的肾脏活检和血清学测试，患者被诊断为快速进行性肾小球肾炎和系统性红斑狼疮。患者的免疫球蛋白循环抗体标记盐裂皮肤的真皮底部，并在免疫印迹研究中识别III型胶原。虽然甲基强的松龙冲击治疗肾小球肾炎并不能减轻水泡小球样皮疹，但氨苯松龙治疗后皮损显著消失。因亨氏小体形成溶血而停止氨苯砜治疗并未加重大疱性疾病。我们的病例表明，泛发性水泡性丘疹可以是系统性红斑狼疮的唯一表现。它还强调了BSLE与狼疮性肾炎之间的密切时间关系。
Bullous systemic lupus erythematosus (BSLE) is a rare subset of systemic lupus erythematosus that is often associated with autoimmunity to type VII collagen. We describe a 45-year-old woman with BSLE who presented with vesiculobullous lesions as an initial manifestation of SLE. The patient first noticed a widespread urticarial, erythematous eruption associated with tense blisters, erosions, and crusting. She was diagnosed with bullous pemphigoid and underwent a one-month course of treatment with betamethazone. Because of the appearance of marked proteinuria, a subsequent renal biopsy, and serological tests, the patient was diagnosed with rapidly progressive glomerulonephritis and systemic lupus erythematosus. The patient's IgG circulating antibodies labeled the dermal floor of salt-split skin and recognized type VII collagen in immunoblot studies. Although methylprednisolone pulse therapy for glomerulonephritis did not alleviate the vesicullobullous eruption, treatment with dapsone resulted in dramatic disappearance of the lesions. Cessation of dapsone therapy due to hemolysis with Heinz-body formation did not aggravate the bullous disease. Our case illustrates that a generalized vesiculobullous eruption can be the sole presenting manifestation of SLE. It also emphasizes the close temporal relationship between BSLE and lupus nephritis.