Atypical pemphigus: autoimmunity against desmocollins and other non-desmoglein autoantigens

Gualtieri, Bruno; Marzano, Valerio; Grando, Sergei A.

doi:10.23736/s2784-8671.20.06619-5

Cited by 6 publications

(5 citation statements)

References 71 publications

(95 reference statements)

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“…Factors such as low autoantibody titres or recognition of a different keratinocyte autoantigen with a low expression in the utilised substrates could be hypothesised. Indeed, about 10–15% of people with PF or pemphigus vulgaris have undetectable anti‐DSG1 and/or anti‐DSG3 autoantibodies, and possess autoantibodies targeting various autoantigens such as DSC1, DSC3, plakophilin 3, plakoglobin, E‐cadherin, M 3 acetylcholine receptor, SPCA1 and/or various mitochondrial proteins 30 . Pathogenicity of some of these autoantibodies have been demonstrated by various in vitro and in vivo experiments such as antigen‐specific immunoadsorption and subsequent prevention of blister formation and/or simple passive transfer to a neonatal mouse (reviewed previously 30 ).…”

Section: Discussionmentioning

confidence: 99%

“…Indeed, about 10–15% of people with PF or pemphigus vulgaris have undetectable anti‐DSG1 and/or anti‐DSG3 autoantibodies, and possess autoantibodies targeting various autoantigens such as DSC1, DSC3, plakophilin 3, plakoglobin, E‐cadherin, M 3 acetylcholine receptor, SPCA1 and/or various mitochondrial proteins 30 . Pathogenicity of some of these autoantibodies have been demonstrated by various in vitro and in vivo experiments such as antigen‐specific immunoadsorption and subsequent prevention of blister formation and/or simple passive transfer to a neonatal mouse (reviewed previously 30 ). In the present study, the only two proteins overexpressed for the purpose of autoantibody detection were DSC1 and DSG1, proteins considered to be major and minor canine PF antigens, respectively 10,29 .…”

Section: Discussionmentioning

confidence: 99%

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Trunk‐dominant and classic facial pemphigus foliaceus in dogs – comparison of anti‐desmocollin‐1 and anti‐desmoglein‐1 autoantibodies and clinical presentations

Bizikova

Linder

Mamo

2022

Veterinary Dermatology

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Background: Canine trunk-dominant pemphigus foliaceus (PF) is mentioned rarely in the literature.Hypothesis/Objectives: The goal of this study was to provide clinical description of trunk-dominant PF and to demonstrate the prevalence of serum antikeratinocyte, anti-desmocollin-1 (DSC1) and anti-desmoglein-1 (DSG1) antibodies, and determine their diagnostic value in this particular PF phenotype.Materials and methods: Clinically relevant information was collected from 31, 25 and 34 dogs with trunk-dominant and facial PF and superficial pyoderma (SP), respectively. Sera from these dogs were tested for antikeratinocyte, anti-DSC1 and anti-DSG1 antibodies using indirect immunofluorescence on canine tissues and DSC1-and DSG1-transfected cells. Sera from healthy dogs and dogs with clinically irrelevant diseases served as controls.Results: Footpad involvement and grouped/polycyclic lesion organisation were identified as features of both PF phenotypes, and not of SP.Antikeratinocyte immunoglobulin (Ig)G was not specific for canine PF. By contrast, antigen-specific IgG was detected only in PF sera; anti-DSC1 IgG in 100% and 58% of dogs with facial and trunk-dominant PF, respectively, and anti-DSG1 IgG in 7% of dogs with trunk-dominant PF only. Conclusions:Trunk-dominant PF shares DSC1 as a major autoantigen with facial PF. The ability to detect anti-DSC1 IgG is lower in trunk-dominant PF, yet despite the lower sensitivity, the positive predictive value and accuracy of this particular anti-DSC1 IgG test are high. A negative test result, however, cannot exclude the diagnosis, and characteristic clinical features such as footpad involvement and/or grouped/polycyclic lesions must be considered when distinguishing trunk-dominant PF from its most relevant differential diagnosis: SP.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Trunk‐dominant and classic facial pemphigus foliaceus in dogs – comparison of anti‐desmocollin‐1 and anti‐desmoglein‐1 autoantibodies and clinical presentations

Bizikova

Linder

Mamo

2022

Veterinary Dermatology

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“…Нарушения адгезивной функции кератиноцитов, вызванные единственным антителом, способны самовосстанавливаться. Атипичная пузырчатка представляет собой уникальную модель для выяснения молекулярных механизмов аутоиммунитета против антигенов, не относящихся к десмоглеину [33].…”

Section: десмоглеиныunclassified

Immunopathology of acantholytic pemphigus (review)

Khamaganova¹,

Ermolaeva²

2022

Immunopathol Allergol Infectol

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The review presents problems of immunopathology of acantholytic pemphigus. Simultaneous increase of antibodies to desmoglein-1 and desmoglein-3 is revealed in pemphigus vulgaris and in pemphigus vegetans. In vulgar pemphigus with prevailing mucosal lesions the erosions appeared on the oral mucosa where desmoglein-3 was prevalent. The blister formation does not need complement activation as in other dermatoses, for the presence of framents of monoclonal antibody is sufficient. In pemphigus autoantibodies relate mainly to IgG4 subclass which does not activate complement, poorly activate effector cells through the Fc region and ineffectively crosslinks the antigen, which directly influences the desmosomal functions. In the case of atypical acantholytic pemphigus, non–desmoglein autoantigens may be revealed.

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“…However, up to 15% of patients with acute PV do not have anti-Dsg1/3 antibodies, determined using ELISA [ 8 ]. More recently, autoantibodies against Dsc1 and Dsc3, mitochondrial proteins, human leukocyte antigens, molecules, thyroid peroxidase (TPO), a Ca 2+ /Mn 2+ -ATPase encoded by ATP2C1 (hSPCA1), and several muscarinic and nicotinic AChRs have been identified as novel targets for pemphigus autoimmunity [ 6 , 9 ].…”

Section: Introductionmentioning

confidence: 99%

The Role of Non-Neuronal Acetylcholine in the Autoimmune Blistering Disease Pemphigus Vulgaris

Foulad

Cirillo

Grando

2023

Biology

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The importance of acetylcholine (ACh) in keratinocyte adhesion and acantholysis has been investigated over the last three decades, particularly in the pathophysiology of autoimmune blistering dermatoses. Pemphigus vulgaris (PV) is an autoimmune blistering skin disease where autoantibody-mediated suprabasilar intraepidermal splitting causes flaccid blisters and non-healing erosions of the oral mucosa and sometimes also of the skin. Historically, acantholysis in PV was thought to be driven by anti-desmoglein (Dsg) antibodies. Herein, we describe the role of autoantibodies against keratinocyte muscarinic and nicotinic acetylcholine receptors, as well as the annexin-like molecule pemphaxin that also binds ACh, in the immunopathogenesis of PV. The identification of targets in this disease is important, as they may lead to novel diagnostic and therapeutic options in the future for this potentially deadly disease.

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Atypical pemphigus: autoimmunity against desmocollins and other non-desmoglein autoantigens

Abstract: The final version may contain major or minor changes.Subscription: Information about subscribing to Minerva Medica journals is online at: http://www.minervamedica.it/en/how-to-order-journals.php Reprints and permissions: For information about reprints and permissions send an email to:

Cited by 6 publications

References 71 publications

Trunk‐dominant and classic facial pemphigus foliaceus in dogs – comparison of anti‐desmocollin‐1 and anti‐desmoglein‐1 autoantibodies and clinical presentations

Trunk‐dominant and classic facial pemphigus foliaceus in dogs – comparison of anti‐desmocollin‐1 and anti‐desmoglein‐1 autoantibodies and clinical presentations

Immunopathology of acantholytic pemphigus (review)

The Role of Non-Neuronal Acetylcholine in the Autoimmune Blistering Disease Pemphigus Vulgaris

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