Alterations of Total Serum Immunoglobulin Concentrations in Pemphigus and Pemphigoid: Selected IgG2 Deficiency in Bullous Pemphigoid

Pemphigus and pemphigoid diseases are organ-specific autoimmune diseases of the skin and/or mucous membranes, which are caused by autoantibodies targeting structural proteins of the skin. In other autoimmune diseases, a high prevalence of primary antibody deficiencies was noted. Conversely, a high p...

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Main Authors: Stanislav Khil'chenko, Katharina Boch, Nina van Beek, Artem Vorobyev, Detlef Zillikens, Enno Schmidt, Ralf J. Ludwig
Format: Article
Language:English
Published: Frontiers Media S.A. 2020-09-01
Series:Frontiers in Medicine
Subjects:
Online Access:https://www.frontiersin.org/article/10.3389/fmed.2020.00472/full
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author Stanislav Khil'chenko
Stanislav Khil'chenko
Katharina Boch
Katharina Boch
Nina van Beek
Nina van Beek
Artem Vorobyev
Artem Vorobyev
Detlef Zillikens
Detlef Zillikens
Enno Schmidt
Enno Schmidt
Enno Schmidt
Ralf J. Ludwig
Ralf J. Ludwig
author_facet Stanislav Khil'chenko
Stanislav Khil'chenko
Katharina Boch
Katharina Boch
Nina van Beek
Nina van Beek
Artem Vorobyev
Artem Vorobyev
Detlef Zillikens
Detlef Zillikens
Enno Schmidt
Enno Schmidt
Enno Schmidt
Ralf J. Ludwig
Ralf J. Ludwig
author_sort Stanislav Khil'chenko
collection DOAJ
description Pemphigus and pemphigoid diseases are organ-specific autoimmune diseases of the skin and/or mucous membranes, which are caused by autoantibodies targeting structural proteins of the skin. In other autoimmune diseases, a high prevalence of primary antibody deficiencies was noted. Conversely, a high prevalence of autoimmune diseases is reported in patients with primary antibody deficiencies. With the exception of one study, pointing toward a decrease of IgG in pemphigus patients, with a relative enrichment of IgG4, serum immunoglobulin (Ig) concentrations had not been studied in pemphigus and pemphigoid. Hence, we here aimed to investigate serum concentrations of IgM, IgA, IgG, and IgG1–4 in pemphigus and pemphigoid patients, as well as in healthy controls. Serum Ig concentrations were determined by ELISA in 105 healthy controls, 100 pemphigus vulgaris (PV), 100 pemphigus foliaceus, 99 bullous pemphigoid (BP), and 55 linear IgA bullous dermatosis (LAD) patients. In healthy controls, age had a significant impact on Ig serum concentrations: In controls at ages of 69 years or older, IgM and IgG were decreased, while all other Ig, except IgA and IgG4, were increased. When stratified by sex, lower IgM concentrations were observed in males. When corrected for age and/or sex, and compared to controls, an increase in serum IgA was noted in LAD. In almost all patient cohorts, an increase in IgG1 and IgG4 was observed, while a decrease in IgG2 or IgG3 was seen in BP or PV patients. This points toward a possible association of BP with IgG2 deficiency and warrants evaluation of IgG2 in BP patients prior to immunosuppressive therapy.
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spelling doaj.art-910bf8a9949a4324ae13fc979af85ca02022-12-22T00:16:26ZengFrontiers Media S.A.Frontiers in Medicine2296-858X2020-09-01710.3389/fmed.2020.00472545281Alterations of Total Serum Immunoglobulin Concentrations in Pemphigus and Pemphigoid: Selected IgG2 Deficiency in Bullous PemphigoidStanislav Khil'chenko0Stanislav Khil'chenko1Katharina Boch2Katharina Boch3Nina van Beek4Nina van Beek5Artem Vorobyev6Artem Vorobyev7Detlef Zillikens8Detlef Zillikens9Enno Schmidt10Enno Schmidt11Enno Schmidt12Ralf J. Ludwig13Ralf J. Ludwig14Lübeck Institute of Experimental Dermatology, University of Lübeck, Lübeck, GermanyCenter for Research on Inflammation of the Skin, University of Lübeck, Lübeck, GermanyCenter for Research on Inflammation of the Skin, University of Lübeck, Lübeck, GermanyDepartment of Dermatology, University of Lübeck, Lübeck, GermanyCenter for Research on Inflammation of the Skin, University of Lübeck, Lübeck, GermanyDepartment of Dermatology, University of Lübeck, Lübeck, GermanyCenter for Research on Inflammation of the Skin, University of Lübeck, Lübeck, GermanyDepartment of Dermatology, University of Lübeck, Lübeck, GermanyCenter for Research on Inflammation of the Skin, University of Lübeck, Lübeck, GermanyDepartment of Dermatology, University of Lübeck, Lübeck, GermanyLübeck Institute of Experimental Dermatology, University of Lübeck, Lübeck, GermanyCenter for Research on Inflammation of the Skin, University of Lübeck, Lübeck, GermanyDepartment of Dermatology, University of Lübeck, Lübeck, GermanyLübeck Institute of Experimental Dermatology, University of Lübeck, Lübeck, GermanyCenter for Research on Inflammation of the Skin, University of Lübeck, Lübeck, GermanyPemphigus and pemphigoid diseases are organ-specific autoimmune diseases of the skin and/or mucous membranes, which are caused by autoantibodies targeting structural proteins of the skin. In other autoimmune diseases, a high prevalence of primary antibody deficiencies was noted. Conversely, a high prevalence of autoimmune diseases is reported in patients with primary antibody deficiencies. With the exception of one study, pointing toward a decrease of IgG in pemphigus patients, with a relative enrichment of IgG4, serum immunoglobulin (Ig) concentrations had not been studied in pemphigus and pemphigoid. Hence, we here aimed to investigate serum concentrations of IgM, IgA, IgG, and IgG1–4 in pemphigus and pemphigoid patients, as well as in healthy controls. Serum Ig concentrations were determined by ELISA in 105 healthy controls, 100 pemphigus vulgaris (PV), 100 pemphigus foliaceus, 99 bullous pemphigoid (BP), and 55 linear IgA bullous dermatosis (LAD) patients. In healthy controls, age had a significant impact on Ig serum concentrations: In controls at ages of 69 years or older, IgM and IgG were decreased, while all other Ig, except IgA and IgG4, were increased. When stratified by sex, lower IgM concentrations were observed in males. When corrected for age and/or sex, and compared to controls, an increase in serum IgA was noted in LAD. In almost all patient cohorts, an increase in IgG1 and IgG4 was observed, while a decrease in IgG2 or IgG3 was seen in BP or PV patients. This points toward a possible association of BP with IgG2 deficiency and warrants evaluation of IgG2 in BP patients prior to immunosuppressive therapy.https://www.frontiersin.org/article/10.3389/fmed.2020.00472/fullpemphiguspemphigoidimmunoglobulinIgGIgMIgA
spellingShingle Stanislav Khil'chenko
Stanislav Khil'chenko
Katharina Boch
Katharina Boch
Nina van Beek
Nina van Beek
Artem Vorobyev
Artem Vorobyev
Detlef Zillikens
Detlef Zillikens
Enno Schmidt
Enno Schmidt
Enno Schmidt
Ralf J. Ludwig
Ralf J. Ludwig
Alterations of Total Serum Immunoglobulin Concentrations in Pemphigus and Pemphigoid: Selected IgG2 Deficiency in Bullous Pemphigoid
Frontiers in Medicine
pemphigus
pemphigoid
immunoglobulin
IgG
IgM
IgA
title Alterations of Total Serum Immunoglobulin Concentrations in Pemphigus and Pemphigoid: Selected IgG2 Deficiency in Bullous Pemphigoid
title_full Alterations of Total Serum Immunoglobulin Concentrations in Pemphigus and Pemphigoid: Selected IgG2 Deficiency in Bullous Pemphigoid
title_fullStr Alterations of Total Serum Immunoglobulin Concentrations in Pemphigus and Pemphigoid: Selected IgG2 Deficiency in Bullous Pemphigoid
title_full_unstemmed Alterations of Total Serum Immunoglobulin Concentrations in Pemphigus and Pemphigoid: Selected IgG2 Deficiency in Bullous Pemphigoid
title_short Alterations of Total Serum Immunoglobulin Concentrations in Pemphigus and Pemphigoid: Selected IgG2 Deficiency in Bullous Pemphigoid
title_sort alterations of total serum immunoglobulin concentrations in pemphigus and pemphigoid selected igg2 deficiency in bullous pemphigoid
topic pemphigus
pemphigoid
immunoglobulin
IgG
IgM
IgA
url https://www.frontiersin.org/article/10.3389/fmed.2020.00472/full
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