Direct Immunofluorescence Using Non-Lesional Buccal Mucosa in Mucous Membrane Pemphigoid

Mucous membrane pemphigoid (MMP) is a rare organ-specific autoimmune subepithelial blistering disease with predominantly mucosal erosions, most frequently affecting the gingiva. Erosions in the oral cavity usually result in markedly decreased quality of life. The major autoantigens are BP180 and lam...

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Main Authors: Mayumi Kamaguchi, Hiroaki Iwata, Inkin Ujiie, Hideyuki Ujiie, Jun Sato, Yoshimasa Kitagawa, Hiroshi Shimizu
Format: Article
Language:English
Published: Frontiers Media S.A. 2018-02-01
Series:Frontiers in Medicine
Subjects:
Online Access:http://journal.frontiersin.org/article/10.3389/fmed.2018.00020/full
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author Mayumi Kamaguchi
Mayumi Kamaguchi
Hiroaki Iwata
Inkin Ujiie
Hideyuki Ujiie
Jun Sato
Yoshimasa Kitagawa
Hiroshi Shimizu
author_facet Mayumi Kamaguchi
Mayumi Kamaguchi
Hiroaki Iwata
Inkin Ujiie
Hideyuki Ujiie
Jun Sato
Yoshimasa Kitagawa
Hiroshi Shimizu
author_sort Mayumi Kamaguchi
collection DOAJ
description Mucous membrane pemphigoid (MMP) is a rare organ-specific autoimmune subepithelial blistering disease with predominantly mucosal erosions, most frequently affecting the gingiva. Erosions in the oral cavity usually result in markedly decreased quality of life. The major autoantigens are BP180 and laminin332, which are components of basement membrane proteins in the skin and mucosa. Diagnosis is usually difficult due to histological destruction of the tissue and low autoantibody titers. In this study, we evaluated the diagnostic value of direct immunofluorescence (DIF) using non-lesional buccal mucosa in seven cases of MMP. In all seven patients, gingival lesions were clinically observed, and in one of the seven patients, buccal lesions were also clinically observed. First, we performed DIF to detect tissue-bound autoantibodies and complement. DIF from non-lesional buccal mucosa revealed linear deposits of IgG and C3 at the basement membrane zone in all cases. To detect autoantibodies, indirect immunofluorescence (IIF), BP180-NC16A ELISA and immunoblotting were performed. Surprisingly, circulating autoantibodies were unable to be detected in any of the cases by ELISA, IIF, or immunoblotting. Furthermore, histological separation was observed in one patient. In conclusion, DIF using non-lesional buccal mucosa was found to be superior to histological and serological tests for diagnosing mucous membrane pemphigoid. The procedure is technically easy and has high diagnostic value.
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spelling doaj.art-fb5c6ed7e0f44db8865cb7bfaf7284622022-12-21T18:23:28ZengFrontiers Media S.A.Frontiers in Medicine2296-858X2018-02-01510.3389/fmed.2018.00020337869Direct Immunofluorescence Using Non-Lesional Buccal Mucosa in Mucous Membrane PemphigoidMayumi Kamaguchi0Mayumi Kamaguchi1Hiroaki Iwata2Inkin Ujiie3Hideyuki Ujiie4Jun Sato5Yoshimasa Kitagawa6Hiroshi Shimizu7Department of Dermatology, Hokkaido University Graduate School of Medicine, Sapporo, JapanDepartment of Oral Diagnosis and Medicine, Hokkaido University Graduate School of Dental Medicine, Sapporo, JapanDepartment of Dermatology, Hokkaido University Graduate School of Medicine, Sapporo, JapanDepartment of Dermatology, Hokkaido University Graduate School of Medicine, Sapporo, JapanDepartment of Dermatology, Hokkaido University Graduate School of Medicine, Sapporo, JapanDepartment of Oral Diagnosis and Medicine, Hokkaido University Graduate School of Dental Medicine, Sapporo, JapanDepartment of Oral Diagnosis and Medicine, Hokkaido University Graduate School of Dental Medicine, Sapporo, JapanDepartment of Dermatology, Hokkaido University Graduate School of Medicine, Sapporo, JapanMucous membrane pemphigoid (MMP) is a rare organ-specific autoimmune subepithelial blistering disease with predominantly mucosal erosions, most frequently affecting the gingiva. Erosions in the oral cavity usually result in markedly decreased quality of life. The major autoantigens are BP180 and laminin332, which are components of basement membrane proteins in the skin and mucosa. Diagnosis is usually difficult due to histological destruction of the tissue and low autoantibody titers. In this study, we evaluated the diagnostic value of direct immunofluorescence (DIF) using non-lesional buccal mucosa in seven cases of MMP. In all seven patients, gingival lesions were clinically observed, and in one of the seven patients, buccal lesions were also clinically observed. First, we performed DIF to detect tissue-bound autoantibodies and complement. DIF from non-lesional buccal mucosa revealed linear deposits of IgG and C3 at the basement membrane zone in all cases. To detect autoantibodies, indirect immunofluorescence (IIF), BP180-NC16A ELISA and immunoblotting were performed. Surprisingly, circulating autoantibodies were unable to be detected in any of the cases by ELISA, IIF, or immunoblotting. Furthermore, histological separation was observed in one patient. In conclusion, DIF using non-lesional buccal mucosa was found to be superior to histological and serological tests for diagnosing mucous membrane pemphigoid. The procedure is technically easy and has high diagnostic value.http://journal.frontiersin.org/article/10.3389/fmed.2018.00020/fullautoimmune diseasedirect immunofluorescencemucous membrane pemphigoidoral mucosaautoantibody
spellingShingle Mayumi Kamaguchi
Mayumi Kamaguchi
Hiroaki Iwata
Inkin Ujiie
Hideyuki Ujiie
Jun Sato
Yoshimasa Kitagawa
Hiroshi Shimizu
Direct Immunofluorescence Using Non-Lesional Buccal Mucosa in Mucous Membrane Pemphigoid
Frontiers in Medicine
autoimmune disease
direct immunofluorescence
mucous membrane pemphigoid
oral mucosa
autoantibody
title Direct Immunofluorescence Using Non-Lesional Buccal Mucosa in Mucous Membrane Pemphigoid
title_full Direct Immunofluorescence Using Non-Lesional Buccal Mucosa in Mucous Membrane Pemphigoid
title_fullStr Direct Immunofluorescence Using Non-Lesional Buccal Mucosa in Mucous Membrane Pemphigoid
title_full_unstemmed Direct Immunofluorescence Using Non-Lesional Buccal Mucosa in Mucous Membrane Pemphigoid
title_short Direct Immunofluorescence Using Non-Lesional Buccal Mucosa in Mucous Membrane Pemphigoid
title_sort direct immunofluorescence using non lesional buccal mucosa in mucous membrane pemphigoid
topic autoimmune disease
direct immunofluorescence
mucous membrane pemphigoid
oral mucosa
autoantibody
url http://journal.frontiersin.org/article/10.3389/fmed.2018.00020/full
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