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Bullous pemphigoid antigen II (BP180) and its soluble extracellular domains are major autoantigens in mucous membrane pemphigoid: the pathogenic relevance to HLA class II alleles and disease severity.大疱性类天疱疮抗原II（BP180）及其可溶性细胞外结构域是黏膜类天疱疮中的主要自身抗原：与HLA II类等位基因及疾病严重程度的致病相关性。

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Linear IgA dermatosis adult variant with oral manifestation: A rare case report.成人型线状IgA大疱性皮病伴口腔表现：1例罕见病例报告

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本文引用的文献

1

Linear IgA dermatosis: report of an infantile case and analysis of 213 cases in Japan.线状IgA大疱性皮病：1例婴儿病例报告及日本213例病例分析

J Dermatol. 2008 Nov;35(11):737-43. doi: 10.1111/j.1346-8138.2008.00561.x.

2

IgA autoantibodies against the NC16a domain of BP180 but not 120-kDa LAD-1 detected in a patient with linear IgA disease.在一名线性IgA疾病患者中检测到针对BP180的NC16a结构域而非120-kDa LAD-1的IgA自身抗体。

Br J Dermatol. 2008 May;158(5):1151-3. doi: 10.1111/j.1365-2133.2008.08492.x. Epub 2008 Mar 20.

3

Localized linear IgA disease induced by ampicillin/sulbactam.

J Am Acad Dermatol. 2004 Jul;51(1):95-8. doi: 10.1016/j.jaad.2004.03.027.

4

Chronic bullous dermatosis of childhood.儿童慢性大疱性皮肤病

Postgrad Med J. 2003 Aug;79(934):437. doi: 10.1136/pmj.79.934.437.

5

The shed ectodomain of collagen XVII/BP180 is targeted by autoantibodies in different blistering skin diseases.在不同的水疱性皮肤病中，自身抗体可靶向攻击 XVII 型胶原蛋白/ BP180 的脱落细胞外结构域。

Am J Pathol. 2000 Feb;156(2):685-95. doi: 10.1016/S0002-9440(10)64772-4.

6

Autoantibodies in a subgroup of patients with linear IgA disease react with the NC16A domain of BP1801.线状IgA疾病患者亚组中的自身抗体与BP1801的NC16A结构域发生反应。

J Invest Dermatol. 1999 Dec;113(6):947-53. doi: 10.1046/j.1523-1747.1999.00808.x.

7

BP180/type XVII collagen: its role in acquired and inherited disorders or the dermal-epidermal junction.BP180/ XVII型胶原蛋白：其在获得性和遗传性皮肤-表皮连接障碍中的作用

Arch Dermatol Res. 1999 Apr;291(4):187-94. doi: 10.1007/s004030050392.

8

Multiple blisters in a young boy. Linear IgA disease of childhood (LADC). (Chronic bullous dermatosis of childhood).

Arch Dermatol. 1998 May;134(5):625, 628. doi: 10.1001/archderm.134.5.625.

9

Linear IgA bullous dermatosis of childhood (chronic bullous dermatosis of childhood).

Clin Dermatol. 1991 Jul-Sep;9(3):393-401. doi: 10.1016/0738-081x(91)90031-f.

10

IgA linear dermatosis of childhood (chronic bullous disease of childhood).儿童IgA线性皮肤病（儿童慢性大疱性疾病）。

Br J Dermatol. 1979 Nov;101(5):535-42. doi: 10.1111/j.1365-2133.1979.tb11882.x.

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一例对120 kDa抗原（LAD-1）产生反应的儿童慢性大疱性疾病。

A Case of Chronic Bullous Disease of Childhood That Was Reactive to the Antigen of 120 kDa (LAD-1).

作者信息

Han Jung Hyun, Yun Sook Jung, Kim Seong-Jin, Lee Seung-Chul, Won Young Ho, Lee Jee-Bum

机构信息

Department of Dermatology, Chonnam National University Medical School, Gwangju, Korea.

出版信息

Ann Dermatol. 2011 May;23(2):209-12. doi: 10.5021/ad.2011.23.2.209. Epub 2011 May 27.

DOI:10.5021/ad.2011.23.2.209

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC3130866/

Abstract

Chronic bullous disease of childhood (CBDC) is an autoimmune blistering disease that is characterized by Immunoglobulin A (IgA) deposits at the basement membrane zone. IgA autoantibodies (aAbs) from the serum of patients with CBDC react with antigens of 97 kDa (LABD97) and 120 kDa (LAD-1), and both of which are fragments of the extracellular domain of bullous pemphigoid 180 (BP180, type XVII collagen). The CBDC sera reacts with the immunodominant NC16a domain of BP180, which is the major region recognized by IgG aAbs in patients with bullous pemphigoid. A five-year-old boy presented with multiple pruritic tense blisters on the umbilical and inguinal areas for six weeks. The direct immunofluorescence of the perilesional area demonstrated linear deposits of IgA at the basement membrane zone. Using immunoblotting and an enzyme linked immunosorbent assay (ELISA), we identified the IgA aAbs reactive to antigens with a molecular weight of 120 kDa (LAD-1), which is a fragment of the extracellular domain of BP180.

摘要

儿童慢性大疱性疾病（CBDC）是一种自身免疫性水疱病，其特征是免疫球蛋白A（IgA）沉积于基底膜带。CBDC患者血清中的IgA自身抗体（aAbs）与97 kDa（LABD97）和120 kDa（LAD-1）的抗原发生反应，这两种抗原均为大疱性类天疱疮180（BP180，XVII型胶原）细胞外结构域的片段。CBDC血清与BP180的免疫显性NC16a结构域发生反应，该结构域是大疱性类天疱疮患者中IgG aAbs识别的主要区域。一名5岁男孩脐部和腹股沟区出现多发瘙痒性紧张水疱6周。皮损周围区域的直接免疫荧光显示基底膜带处有IgA线性沉积。通过免疫印迹和酶联免疫吸附测定（ELISA），我们鉴定出与分子量为120 kDa的抗原（LAD-1）反应的IgA aAbs，LAD-1是BP180细胞外结构域的一个片段。