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静脉注射免疫球蛋白治疗儿童大疱性类天疱疮。

Childhood bullous pemphigoid treated by i.v. immunoglobulin.

作者信息

Xiao Ting, Li Bo, Wang Ya-Kun, He Chun-Di, Chen Hong-Duo

机构信息

Department of Dermatology, No. 1 Hospital of China Medical University, Shenyang, China.

出版信息

J Dermatol. 2007 Sep;34(9):650-3. doi: 10.1111/j.1346-8138.2007.00348.x.

Abstract

Bullous pemphigoid is an acquired autoimmune subepidermal blistering disorder mostly seen in the elderly. Childhood bullous pemphigoid is very rare. For the first time we report a case of childhood bullous pemphigoid associated with infantile eczema. Two weeks after a routine vaccination, a 3.5-month-old boy with infantile eczema developed a generalized blistering disorder. Histopathology revealed a subepidermal blister. Direct immunofluorescence showed linear depositions of C3 along the basement membrane zone. Indirect immunofluorescence studies demonstrated the presence of circulating immunoglobulin G antibodies directed against the epidermal side of salt-split skin. Enzyme-linked immunosorbent assay demonstrated serum level of anti-BP180 antibody elevated. The patient was successfully treated by high-dose i.v. immunoglobulin.

摘要

大疱性类天疱疮是一种获得性自身免疫性表皮下大疱性疾病,多见于老年人。儿童大疱性类天疱疮非常罕见。我们首次报告一例与婴儿湿疹相关的儿童大疱性类天疱疮病例。一名3.5个月大患婴儿湿疹的男孩在常规疫苗接种两周后出现全身性大疱性疾病。组织病理学显示为表皮下大疱。直接免疫荧光显示C3沿基底膜带呈线性沉积。间接免疫荧光研究表明存在针对盐裂皮肤表皮侧的循环免疫球蛋白G抗体。酶联免疫吸附测定显示抗BP180抗体血清水平升高。该患者通过大剂量静脉注射免疫球蛋白成功治愈。

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