Clinical and Immunological Study of 30 Cases With Both IgG and IgA Anti-Keratinocyte Cell Surface Autoantibodies Toward the Definition of Intercellular IgG/IgA Dermatosis

Several sporadic cases, in which direct and indirect immunofluorescence studies simultaneously detected IgG and IgA autoantibodies to keratinocyte cell surfaces, have been reported mainly under the name of IgG/IgA pemphigus. However, there have been no systematic studies for this condition. In this...

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Main Authors: Takashi Hashimoto, Kwesi Teye, Koji Hashimoto, Katarzyna Wozniak, Daisuke Ueo, Sakuhei Fujiwara, Kazuhiro Inafuku, Yorihisa Kotobuki, Ines Lakos Jukic, Branka Marinović, Anna Bruckner, Daisuke Tsuruta, Tamihiro Kawakami, Norito Ishii
Format: Article
Language:English
Published: Frontiers Media S.A. 2018-05-01
Series:Frontiers in Immunology
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Online Access:http://journal.frontiersin.org/article/10.3389/fimmu.2018.00994/full
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author Takashi Hashimoto
Takashi Hashimoto
Kwesi Teye
Koji Hashimoto
Katarzyna Wozniak
Daisuke Ueo
Sakuhei Fujiwara
Kazuhiro Inafuku
Yorihisa Kotobuki
Ines Lakos Jukic
Branka Marinović
Anna Bruckner
Daisuke Tsuruta
Tamihiro Kawakami
Norito Ishii
author_facet Takashi Hashimoto
Takashi Hashimoto
Kwesi Teye
Koji Hashimoto
Katarzyna Wozniak
Daisuke Ueo
Sakuhei Fujiwara
Kazuhiro Inafuku
Yorihisa Kotobuki
Ines Lakos Jukic
Branka Marinović
Anna Bruckner
Daisuke Tsuruta
Tamihiro Kawakami
Norito Ishii
author_sort Takashi Hashimoto
collection DOAJ
description Several sporadic cases, in which direct and indirect immunofluorescence studies simultaneously detected IgG and IgA autoantibodies to keratinocyte cell surfaces, have been reported mainly under the name of IgG/IgA pemphigus. However, there have been no systematic studies for this condition. In this study, we collected 30 cases of this condition from our cohort of more than 5,000 autoimmune bullous disease cases, which were consulted for our diagnostic methods from other institutes, and summarized their clinical and immunological findings. Clinically, there was no male–female prevalence, mean age of disease onset was 55.6 years, and mean duration before this condition was suspected was 18 months. The patients showed clinically bullous and pustular skin lesions preferentially on the trunk and extremities, and histopathologically intraepidermal pustules and blisters with infiltration of neutrophils and eosinophils. Immunologically, ELISAs frequently detected IgG and IgA autoantibodies to both desmogleins and desmocollins. From the characteristic clinical, histopathological, and immunological features, which are considerably different from those in classical IgG types of pemphigus, we propose this disease as a new disease entity with preferential name of intercellular IgG/IgA dermatosis (IGAD). This was the largest study of IGAD to date.
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spelling doaj.art-84830876defe447cb9f9b157939de9482022-12-22T01:28:53ZengFrontiers Media S.A.Frontiers in Immunology1664-32242018-05-01910.3389/fimmu.2018.00994334774Clinical and Immunological Study of 30 Cases With Both IgG and IgA Anti-Keratinocyte Cell Surface Autoantibodies Toward the Definition of Intercellular IgG/IgA DermatosisTakashi Hashimoto0Takashi Hashimoto1Kwesi Teye2Koji Hashimoto3Katarzyna Wozniak4Daisuke Ueo5Sakuhei Fujiwara6Kazuhiro Inafuku7Yorihisa Kotobuki8Ines Lakos Jukic9Branka Marinović10Anna Bruckner11Daisuke Tsuruta12Tamihiro Kawakami13Norito Ishii14Department of Dermatology, Osaka City University Graduate School of Medicine, Osaka, JapanKurume University School of Medicine, Kurume, JapanKurume University Institute of Cutaneous Cell Biology, Kurume, JapanDepartment of Life Sciences, Graduate School of Arts and Sciences, University of Tokyo, Tokyo, JapanDepartment of Dermatology and Immunodermatology, Medical University of Warsaw, Warsaw, PolandUeo Dermatology Clinic, Saiki, JapanDepartment of Dermatology, Faculty of Medicine, Oita University, Yufu, JapanDepartment of Dermatology, Kimitsu Chuo Hospital, Kimitsu, JapanDepartment of Dermatology, Osaka University Graduate School of Medicine, Suita, Japan0University Hospital Centre Zagreb, Department of Dermatology and Venereology, School of Medicine, University of Zagreb, Zagreb, Croatia0University Hospital Centre Zagreb, Department of Dermatology and Venereology, School of Medicine, University of Zagreb, Zagreb, Croatia1Pediatric Dermatology, University of Colorado School of Medicine, Children’s Hospital Colorado, Denver, CO, United StatesDepartment of Dermatology, Osaka City University Graduate School of Medicine, Osaka, Japan2Department of Dermatology, St. Marriana Medical University, Kawasaki, Japan3Department of Dermatology, Kurume University School of Medicine, Fukuoka, JapanSeveral sporadic cases, in which direct and indirect immunofluorescence studies simultaneously detected IgG and IgA autoantibodies to keratinocyte cell surfaces, have been reported mainly under the name of IgG/IgA pemphigus. However, there have been no systematic studies for this condition. In this study, we collected 30 cases of this condition from our cohort of more than 5,000 autoimmune bullous disease cases, which were consulted for our diagnostic methods from other institutes, and summarized their clinical and immunological findings. Clinically, there was no male–female prevalence, mean age of disease onset was 55.6 years, and mean duration before this condition was suspected was 18 months. The patients showed clinically bullous and pustular skin lesions preferentially on the trunk and extremities, and histopathologically intraepidermal pustules and blisters with infiltration of neutrophils and eosinophils. Immunologically, ELISAs frequently detected IgG and IgA autoantibodies to both desmogleins and desmocollins. From the characteristic clinical, histopathological, and immunological features, which are considerably different from those in classical IgG types of pemphigus, we propose this disease as a new disease entity with preferential name of intercellular IgG/IgA dermatosis (IGAD). This was the largest study of IGAD to date.http://journal.frontiersin.org/article/10.3389/fimmu.2018.00994/fullautoimmune bullous diseasesdesmocollindesmogleinELISAintercellular IgG/IgA dermatosis
spellingShingle Takashi Hashimoto
Takashi Hashimoto
Kwesi Teye
Koji Hashimoto
Katarzyna Wozniak
Daisuke Ueo
Sakuhei Fujiwara
Kazuhiro Inafuku
Yorihisa Kotobuki
Ines Lakos Jukic
Branka Marinović
Anna Bruckner
Daisuke Tsuruta
Tamihiro Kawakami
Norito Ishii
Clinical and Immunological Study of 30 Cases With Both IgG and IgA Anti-Keratinocyte Cell Surface Autoantibodies Toward the Definition of Intercellular IgG/IgA Dermatosis
Frontiers in Immunology
autoimmune bullous diseases
desmocollin
desmoglein
ELISA
intercellular IgG/IgA dermatosis
title Clinical and Immunological Study of 30 Cases With Both IgG and IgA Anti-Keratinocyte Cell Surface Autoantibodies Toward the Definition of Intercellular IgG/IgA Dermatosis
title_full Clinical and Immunological Study of 30 Cases With Both IgG and IgA Anti-Keratinocyte Cell Surface Autoantibodies Toward the Definition of Intercellular IgG/IgA Dermatosis
title_fullStr Clinical and Immunological Study of 30 Cases With Both IgG and IgA Anti-Keratinocyte Cell Surface Autoantibodies Toward the Definition of Intercellular IgG/IgA Dermatosis
title_full_unstemmed Clinical and Immunological Study of 30 Cases With Both IgG and IgA Anti-Keratinocyte Cell Surface Autoantibodies Toward the Definition of Intercellular IgG/IgA Dermatosis
title_short Clinical and Immunological Study of 30 Cases With Both IgG and IgA Anti-Keratinocyte Cell Surface Autoantibodies Toward the Definition of Intercellular IgG/IgA Dermatosis
title_sort clinical and immunological study of 30 cases with both igg and iga anti keratinocyte cell surface autoantibodies toward the definition of intercellular igg iga dermatosis
topic autoimmune bullous diseases
desmocollin
desmoglein
ELISA
intercellular IgG/IgA dermatosis
url http://journal.frontiersin.org/article/10.3389/fimmu.2018.00994/full
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