Chronic pemphigoid gestationis:: Comparative clinical and immunopathological study of 10 patients
Chronic pemphigoid gestationis:: Comparative clinical and immunopathological study of 10 patients
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DOI：
10.1159/000068467
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发表时间：
2003-01-01
期刊：
影响因子：
3.4
通讯作者：
Bonnetblanc, JM
中科院分区：
文献类型：
--
作者：
Boulinguez, S;Bédane, C;Bonnetblanc, JM
背景资料：妊娠类天疱疮（PG）是一种罕见的自身免疫性大疱性疾病，发生在妊娠的最后三个月，通常在分娩后3个月内消退。长期形式的疾病持续超过6个月后交付已被报道为慢性PG. Objective：本研究的目的是比较4例患者的临床和免疫病理学研究结果之间的正常回归的疾病后交付和6例慢性病程。研究方法：通过直接和间接免疫荧光和Western印迹法，对所有患者的临床模式（年龄、粘膜和皮肤受累、产科史、水疱疾病的持续时间和对治疗的反应）进行评估和研究。8例患者进行了研究，免疫电镜（IEM）和3例患者进行了间接IEM。结果如下：慢性PG患者年龄较大，有多胎妊娠史，既往妊娠有PG病史，广泛皮疹和粘膜受累。循环自身抗体的亚类分析显示，除1例病程7年的患者外，所有患者均出现IgG 1抗BP 180反应。直接IEM在6/8例患者中呈阳性，显示透明板的标记，使用胶体金探针的间接IEM证实了靶抗原定位于透明板中锚定丝的近端部分。结论：本研究表明，即使在慢性持久性PG中，IgG 1仍然是IgG的主要亚型。因此，从该系列中无法确定慢性病的生物学和可预测标志物。版权所有（C）2003 S. Karger AG，巴塞尔。
Background: Pemphigoid gestationis (PG) is a rare autoimmune bullous disorder occurring during the last trimester of pregnancy and usually regressive within 3 months after delivery. Prolonged forms of the disease lasting more than 6 months after delivery have been reported as chronic PG. Objective:The aim of the present study was to compare the clinical and immunopathological findings between 4 patients presenting a normal regression of the disease after delivery and 6 patients with a chronic course. Methods: All patients were evaluated and studied by clinical patterns (age, mucosal and cutaneous involvement, obstetrical history, duration of the blistering disease and response to treatment), by direct and indirect immunofluorescence and Western blot. Eight patients were studied by immunoelectron microscopy (IEM) and 3 patients had an indirect IEM. Results: Patients with chronic PG were older, had multi-gravidity, a history of PG during previous pregnancies, widespread cutaneous eruption and mucosal involvement. Subclass analysis of circulating autoantibodies showed an IgG1 anti-BP180 response in all patients except 1 with disease of 7 years' duration. Direct IEM was positive in 6/8 patients showing a labeling of the lamina lucida, and indirect IEM using colloidal gold probes confirmed the localization of the target antigens to the proximal part of the anchoring filaments in the lamina lucida. Conclusion: This study suggests that, even in chronic long-lasting PG, IgG1 remains the predominant subtype of IgG. Therefore no biological and predictable marker of chronicity can be ascertained from this series. Copyright (C) 2003 S. Karger AG, Basel.