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糖皮质激素抵抗性妊娠疱疹:辅助免疫吸附治疗成功。

Glucocorticosteroid-resistant pemphigoid gestationis: successful treatment with adjuvant immunoadsorption.

机构信息

Department of Dermatology, University of Lübeck, Lübeck, Germany.

出版信息

J Dermatol. 2012 Feb;39(2):168-71. doi: 10.1111/j.1346-8138.2011.01376.x.

DOI:10.1111/j.1346-8138.2011.01376.x
PMID:22379622
Abstract

A 40-year old prima para presented with multiple urticaria-like plaques and severe pruritus 2 weeks prior to giving birth by cesarean section. Three days after birth, the disease flared up and tense blisters appeared on hands, lower arms and feet. Based on the clinical presentation, direct immunofluorescence microscopy, complement binding test and detection of high levels of circulating anti-BP180 antibodies, the diagnosis of pemphigoid gestationis was established. Despite treatment with class IV topical corticosteroid and prednisolone p.o. up to 60 mg/day, both skin lesions and severe pruritus progressed accompanied by increasing anti-BP180 antibody serum levels. In order to continue breast feeding, the prednisolone dose could not be further increased and 10 immunoadsorptions over 4 weeks were performed. During this period, skin lesions cleared rapidly, pruritus subsided and BP180-specific serum autoantibodies decreased by 99.5% allowing the reduction of prednisolone to 7.5 mg/day. We conclude that immunoadsorption is an effective and safe adjuvant therapeutic option for severe pemphigoid gestationis.

摘要

一位 40 岁的初产妇,在剖宫产前两周出现多处荨麻疹样斑块和严重瘙痒。分娩后 3 天,疾病发作,手上、小臂和脚上出现紧张性水疱。根据临床表现、直接免疫荧光显微镜检查、补体结合试验和循环中高水平抗 BP180 抗体的检测,诊断为妊娠疱疹。尽管使用了 IV 级局部皮质类固醇和泼尼松龙口服(高达 60mg/天),但皮肤病变和严重瘙痒仍在进展,同时伴有抗 BP180 抗体血清水平的升高。为了继续母乳喂养,无法进一步增加泼尼松龙的剂量,因此在 4 周内进行了 10 次免疫吸附治疗。在此期间,皮肤病变迅速清除,瘙痒缓解,BP180 特异性血清自身抗体下降 99.5%,泼尼松龙剂量降至 7.5mg/天。我们得出结论,免疫吸附是治疗严重妊娠疱疹的一种有效且安全的辅助治疗选择。

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