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线状免疫球蛋白A大疱性皮病:1例成功使用氨苯砜治疗的罕见病例

Linear Immunoglobulin A Dermatosis: A Rare Case Illustrating Successful Treatment With Dapsone.

作者信息

Machado Morais Joana, Meneses Mariana, Freitas Catarina, Oliveira Herberto, Leite Inês

机构信息

Serviço de Pediatria, Hospital Pedro Hispano, Porto, PRT.

Serviço de Anatomia Patológica, Hospital Pedro Hispano, Porto, PRT.

出版信息

Cureus. 2023 Feb 28;15(2):e35569. doi: 10.7759/cureus.35569. eCollection 2023 Feb.

Abstract

This is the case report of a previously healthy four-year-old girl with a history of upper airway infection that was treated with a β-lactam antibiotic. She was seen in the emergency department one month later with vesiculobullous lesions with clear content that were isolated or grouped in rosettes. Direct immunofluorescence showed baseline linear positivity for immunoglobulin A (IgA) (+) and fibrinogen-positive bullous content with absent remaining immunosera expression. The observed results were compatible with linear IgA bullous dermatosis. After confirming the diagnosis and excluding glucose-6-phosphate dehydrogenase (G6PD) deficiency, dapsone was added to the initial treatment with systemic and topical corticosteroids. This case report is a reminder of the importance of a high index of clinical suspicion for this condition to reach a timely diagnosis.

摘要

这是一例先前健康的4岁女童的病例报告,该女童有上呼吸道感染病史,曾接受β-内酰胺类抗生素治疗。一个月后,她在急诊科就诊,身上出现了水疱大疱性皮损,疱液清亮,皮损孤立或呈玫瑰花结状成群分布。直接免疫荧光显示免疫球蛋白A(IgA)基线呈线状阳性(+),疱液中纤维蛋白原阳性,其余免疫血清表达缺失。观察结果符合线状IgA大疱性皮肤病。在确诊并排除葡萄糖-6-磷酸脱氢酶(G6PD)缺乏症后,在初始治疗中加入氨苯砜,同时使用全身性和局部性皮质类固醇。本病例报告提醒我们,对于这种疾病要有高度的临床怀疑指数,以便及时做出诊断。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2ceb/10061548/bb4b906a6ddc/cureus-0015-00000035569-i01.jpg

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