[黏膜类天疱疮：抗BP180抗原的IgG和IgA抗体]

[Mucous membrane pemphigoid: IgG and IgA antibodies against the BP180 antigen].

作者信息

España Agustín, del Olmo Julio, Marquina Miren, Sitaru Cassian

机构信息

Departamento de Dermatología, Clínica Universitaria de Navarra, Pamplona, Spain.

出版信息

Actas Dermosifiliogr. 2005 Jul-Aug;96(6):365-70. doi: 10.1016/s0001-7310(05)73092-1.

DOI:10.1016/s0001-7310(05)73092-1

PMID:16476255

Abstract

INTRODUCTION

Mucous membrane pemphigoid is a group of autoimmune bullous diseases, mediated by autoantibodies directed against different proteins in the dermoepidermal junction, including the BP180 antigen.

PATIENTS, MATERIAL AND METHODS: We included five patients with MMP in this study. We studied the presence of circulating autoantibodies against the BP180 antigen and against recombinant extracellular fragments of this protein.

RESULTS

We detected the presence of circulating antibodies against BP180 in all of the patients. Indirect immunofluorescence (IIF) studies were positive in 2 patients (20 %), as well as in 2 patients via salt-split studies. We found reactivity to the extracellular fragment of BP180 (LAD-1) in 3 patients, 2 of them via IgA and 1 with IgG. The serum of only 2 patients recognized the NC16A fragment, and 4 of the 5 patients had antibodies against the carboxy-terminal domain BP180 4575.

CONCLUSIONS

Molecular biology techniques are very important to complement the diagnosis of MMP, especially when the results of hematoxylin-eosin or IF studies are not satisfactory for a diagnosis of MMP.

摘要

引言

黏膜类天疱疮是一组自身免疫性大疱性疾病，由针对真皮表皮交界处不同蛋白质（包括BP180抗原）的自身抗体介导。

患者、材料与方法：本研究纳入了5例黏膜类天疱疮患者。我们研究了针对BP180抗原以及该蛋白重组细胞外片段的循环自身抗体的存在情况。

结果

我们在所有患者中均检测到了针对BP180的循环抗体。间接免疫荧光（IIF）研究在2例患者（20%）中呈阳性，盐裂皮肤研究在2例患者中呈阳性。我们发现3例患者对BP180（LAD-1）的细胞外片段有反应性，其中2例通过IgA检测到，1例通过IgG检测到。仅2例患者的血清识别NC16A片段，5例患者中有4例具有针对羧基末端结构域BP180 4575的抗体。