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Biomedicines
Special Issues
Autoimmune Blistering Diseases
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Autoimmune Blistering Diseases

A special issue of Biomedicines (ISSN 2227-9059). This special issue belongs to the section "Immunology and Immunotherapy".

Deadline for manuscript submissions: closed (15 December 2022) | Viewed by 16185

Special Issue Editor

Dr. Cezary Kowalewski

E-Mail Website
Guest Editor
Department of Dermatology and Immunodermatology, Medical University of Warsaw, 02-091 Warsaw, Poland
Interests: pemphigus, pemphigoid, mucus membrane pemphigoid, epidermolysis bullosa acquisita, cell-cell and cell-matrix contact proteins, diagnostic applications of laser scanning confocal microscopy, epidermolysis bullosa hereditaria, mesenchymal stem cell in the treatment of EB wounds

Special Issue Information

Dear Colleagues,

Autoimmune blistering diseases are an extremely fascinating field of research. In recent years, most of the adhesion molecules in the epidermis have been characterized, and the pathogenic roles of autoantibodies directed against these antigens in the mechanism of blister formation have been demonstrated in animal models. Concurrently with the study of the humoral immune response, intensive research was carried out on the involvement of cellular immunity and the mechanism of loss of immune tolerance in pemphigus and pemphigoid. The results of these studies give rise to novel therapeutic strategies. Currently, a new challenge in research into the pathogenesis of autoimmune bullous diseases is to investigate the mechanism leading to the development of pemphigus and pemphigoid induced by the administration of biological drugs used in cancer treatment or new antidiabetic drugs. Despite the enormous progress in research, we still do not have sufficient knowledge about the pathogenesis and treatment of mucous membrane pemphigoid and epidermolysis bullosa acquisita—interdisciplinary disease entities with complex pathogenesis. In this Research Topic, we welcome the submission of original research articles and reviews that reflect recent advances in research into various aspects of autoimmune bullous diseases including, but not limited to:

(1)       Identification and characterization of unidentified skin autoantigens;

(2)      Classification of different autoimmune blistering diseases;

(3)      Studies on the pathogenesis of drug-induced autoimmune blistering diseases;

(4)  Studies on pathogenesis of autoimmune blistering diseases using various disease models;
(5)    Novel therapeutic strategies for autoimmune blistering diseases.


Dr. Cezary Kowalewski
Guest Editor

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Biomedicines is an international peer-reviewed open access monthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2600 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • pemphigus
  • paraneoplastic pemphigus
  • pemphigoid
  • mucus membrane pemphigoid
  • epidermolysis bullosa acquisita
  • cell-cell and cell-matrix contact proteins
  • pathogenesis of autoimmune blistering diseases
  • drug induced autoimmune blistering diseases
  • novel therapies of autoimmune blistering diseases

Published Papers (5 papers)

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Research

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16 pages, 11307 KiB  
Article
IgG N-glycosylation from Patients with Pemphigus Treated with Rituximab
by Guillaume Font, Marie-Laure Walet-Balieu, Marie Petit, Carole Burel, Maud Maho-Vaillant, Vivien Hébert, Philippe Chan, Manuel Fréret, Olivier Boyer, Pascal Joly, Sébastien Calbo, Muriel Bardor and Marie-Laure Golinski
Biomedicines 2022, 10(8), 1774; https://0-doi-org.brum.beds.ac.uk/10.3390/biomedicines10081774 - 22 Jul 2022
Cited by 3 | Viewed by 1519
Abstract
Pemphigus is a life-threatening auto-immune blistering disease of the skin and mucous membrane that is caused by the production of auto-antibodies (auto-Abs) directed against adhesion proteins: desmoglein 1 and 3. We demonstrated in the “Ritux3” trial, the high efficacy of rituximab, an anti-CD20 [...] Read more.
Pemphigus is a life-threatening auto-immune blistering disease of the skin and mucous membrane that is caused by the production of auto-antibodies (auto-Abs) directed against adhesion proteins: desmoglein 1 and 3. We demonstrated in the “Ritux3” trial, the high efficacy of rituximab, an anti-CD20 recombinant monoclonal antibody, as the first-line treatment for pemphigus. However, 25% of patients relapsed during the six-month period after rituximab treatment. These early relapses were associated with a lower decrease in anti-desmoglein auto-Abs after the initial cycle of rituximab. The N-glycosylation of immunoglobulin-G (IgG) can affect their affinity for Fc receptors and their serum half-life. We hypothesized that the extended half-life of Abs could be related to modifications of IgG N-glycans. The IgG N-glycome from pemphigus patients and its evolution under rituximab treatment were analyzed. Pemphigus patients presented a different IgG N-glycome than healthy donors, with less galactosylated, sialylated N-glycans, as well as a lower level of N-glycans bearing an additional N-acetylglucosamine. IgG N-glycome from patients who achieved clinical remission was not different to the one observed at baseline. Moreover, our study did not identify the N-glycans profile as discriminating between relapsing and non-relapsing patients. We report that pemphigus patients present a specific IgG N-glycome. The changes observed in these patients could be a biomarker of autoimmunity susceptibility rather than a sign of inflammation. Full article
(This article belongs to the Special Issue Autoimmune Blistering Diseases)
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10 pages, 1758 KiB  
Article
Clinical, Histopathologic, and Immunohistochemical Features of Patients with IgG/IgA Pemphigus
by Yung-Tsu Cho, Ko-Ting Fu, Kai-Lung Chen, Yih-Leong Chang and Chia-Yu Chu
Biomedicines 2022, 10(5), 1197; https://0-doi-org.brum.beds.ac.uk/10.3390/biomedicines10051197 - 22 May 2022
Cited by 1 | Viewed by 4052
Abstract
Pemphigus is an autoantibody-mediated blistering disease. In addition to conventional pemphigus vulgaris and pemphigus foliaceus, several other types have been reported. Among them, IgG/IgA pemphigus is less well defined and seldom reported. To characterize the clinical, histopathologic, and immunohistochemical presentation of IgG/IgA pemphigus, [...] Read more.
Pemphigus is an autoantibody-mediated blistering disease. In addition to conventional pemphigus vulgaris and pemphigus foliaceus, several other types have been reported. Among them, IgG/IgA pemphigus is less well defined and seldom reported. To characterize the clinical, histopathologic, and immunohistochemical presentation of IgG/IgA pemphigus, we retrospectively identified 22 patients with the disease at a referral center in Taiwan. These patients showed two types of skin lesion: annular or arciform erythemas with blisters or erosions (45.5%) and discrete erosions or blisters such as those in conventional pemphigus (54.5%). Mucosal involvement was found in 40.9%. Histopathologic analysis identified acantholysis (77.3%) and intra-epidermal aggregates of neutrophils (40.9%) and eosinophils (31.8%). Direct immunofluorescence studies showed IgG/IgA (100%) and C3 (81.8%) depositions in the intercellular space of the epidermis. In immunohistochemical staining, patients with IgG/IgA pemphigus demonstrated significantly higher levels of epidermal expression of interleukin-8 and matrix metalloproteinase-9 than those with conventional pemphigus (p < 0.05). In conclusion, although IgG/IgA pemphigus is heterogeneous in presentation, it shows characteristic features that are different from other forms of pemphigus and should be considered a distinct type of pemphigus. Full article
(This article belongs to the Special Issue Autoimmune Blistering Diseases)
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15 pages, 1812 KiB  
Article
Longitudinal Pathogenic Properties and N-Glycosylation Profile of Antibodies from Patients with Pemphigus after Corticosteroid Treatment
by Marie Petit, Marie-Laure Walet-Balieu, Damien Schapman, Marie-Laure Golinski, Carole Burel, Marion Barray, Laurent Drouot, Maud Maho-Vaillant, Vivien Hébert, Olivier Boyer, Muriel Bardor, Pascal Joly and Sébastien Calbo
Biomedicines 2021, 9(10), 1411; https://0-doi-org.brum.beds.ac.uk/10.3390/biomedicines9101411 - 08 Oct 2021
Cited by 1 | Viewed by 1549
Abstract
Pemphigus vulgaris is an autoimmune disease that occurs due to pathogenic autoantibodies that recognize the following epidermal adhesion proteins: desmogleins. Systemic corticosteroids usually decrease the titers of anti-desmoglein autoantibodies and improve patients’ conditions. Since modifications of IgG N-glycosylation have been described in [...] Read more.
Pemphigus vulgaris is an autoimmune disease that occurs due to pathogenic autoantibodies that recognize the following epidermal adhesion proteins: desmogleins. Systemic corticosteroids usually decrease the titers of anti-desmoglein autoantibodies and improve patients’ conditions. Since modifications of IgG N-glycosylation have been described in some autoimmune diseases, we hypothesized that changes in the pathogenic activity of pemphigus IgG could be related to changes in their N-glycosylation profile. The purpose of this study was to assess, longitudinally, the pathogenicity of pemphigus serum IgG and their N-glycosylation profile during phases of disease activity and clinical remission. The pathogenic activity of serum IgG was measured in vitro on immortalized keratinocytes, by immunofluorescence and dissociation assays, and IgG N-glycans were analyzed by mass spectrometry. We showed (i) a correlation between pemphigus clinical activity and the pathogenicity of serum IgG at baseline and at month 6, while the persistence of the in vitro pathogenic activity of IgG during its evolution, even in patients in clinical remission, seemed to be predictive of relapse; (ii) that modifications of the N-glycan structure were altered the in vitro pathogenicity of patients’ autoantibodies; (iii) that the pathogenic properties of pemphigus IgG did not appear to be related to the disparity in IgG N-glycans during the course of pemphigus. Full article
(This article belongs to the Special Issue Autoimmune Blistering Diseases)
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Review

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14 pages, 287 KiB  
Review
Pemphigus—The Crux of Clinics, Research, and Treatment during the COVID-19 Pandemic
by Branka Marinović, Joško Miše, Ines Lakoš Jukić and Zrinka Bukvić Mokos
Biomedicines 2021, 9(11), 1555; https://0-doi-org.brum.beds.ac.uk/10.3390/biomedicines9111555 - 28 Oct 2021
Cited by 4 | Viewed by 2192
Abstract
Pemphigus is a rare autoimmune disease characterised by the production of pathogenic autoantibodies in response to different desmosome proteins. The pathophysiological process leads to the development of blisters and erosions on mucosal and/or skin surfaces. The classical clinical variants of pemphigus are pemphigus [...] Read more.
Pemphigus is a rare autoimmune disease characterised by the production of pathogenic autoantibodies in response to different desmosome proteins. The pathophysiological process leads to the development of blisters and erosions on mucosal and/or skin surfaces. The classical clinical variants of pemphigus are pemphigus vulgaris and pemphigus foliaceus. A diagnostic delay is very common in pemphigus, especially among patients with mucosal involvement. However, in recent years we have witnessed considerably fewer patients with extensive mucocutaneous manifestations, since patients with oral lesions are referred to dermatologists to start the treatment much sooner than they had been previously. Among non-classical variants of pemphigus, unusual cases with discrepancies between autoantibody profiles and clinics challenge the “desmoglein compensation theory”. The identification of several other autoantigens that perform a role in the pathogenesis of different variants of pemphigus will progress immunodermatology towards an approach that will determine personalized pemphigus subtypes for each patient. Comorbidities among patients are primarily associated with the prolonged use of corticosteroids and other immunosuppressive agents. The SARS-CoV-2 pandemic raised concerns regarding the immunosuppressive effects of treatment and the risk of a more complicated COVID-19 infection, as well as on the ability to develop an adequate vaccine response. Full article
(This article belongs to the Special Issue Autoimmune Blistering Diseases)
18 pages, 28130 KiB  
Review
Immunotherapeutic Potential of m6A-Modifiers and MicroRNAs in Controlling Acute Myeloid Leukaemia
by Sunil Kumar, Ravinder Nagpal, Amit Kumar, Muhammad Umer Ashraf and Yong-Soo Bae
Biomedicines 2021, 9(6), 690; https://0-doi-org.brum.beds.ac.uk/10.3390/biomedicines9060690 - 18 Jun 2021
Cited by 21 | Viewed by 6031
Abstract
Epigenetic alterations have contributed greatly to human carcinogenesis. Conventional epigenetic studies have been predominantly focused on DNA methylation, histone modifications, and chromatin remodelling. Epitranscriptomics is an emerging field that encompasses the study of RNA modifications that do not affect the RNA sequence but [...] Read more.
Epigenetic alterations have contributed greatly to human carcinogenesis. Conventional epigenetic studies have been predominantly focused on DNA methylation, histone modifications, and chromatin remodelling. Epitranscriptomics is an emerging field that encompasses the study of RNA modifications that do not affect the RNA sequence but affect functionality via a series of RNA binding proteins called writer, reader and eraser. Several kinds of epi-RNA modifications are known, such as 6-methyladenosine (m6A), 5-methylcytidine (m5C), and 1-methyladenosine. M6A modification is the most studied and has large therapeutic implications. In this review, we have summarised the therapeutic potential of m6A-modifiers in controlling haematological disorders, especially acute myeloid leukaemia (AML). AML is a type of blood cancer affecting specific subsets of blood-forming hematopoietic stem/progenitor cells (HSPCs), which proliferate rapidly and acquire self-renewal capacities with impaired terminal cell-differentiation and apoptosis leading to abnormal accumulation of white blood cells, and thus, an alternative therapeutic approach is required urgently. Here, we have described how RNA m6A-modification machineries EEE (Editor/writer: Mettl3, Mettl14; Eraser/remover: FTO, ALKBH5, and Effector/reader: YTHDF-1/2) could be reformed into potential druggable candidates or as RNA-modifying drugs (RMD) to treat leukaemia. Moreover, we have shed light on the role of microRNAs and suppressors of cytokine signalling (SOCS/CISH) in increasing anti-tumour immunity towards leukaemia. We anticipate, our investigation will provide fundamental knowledge in nurturing the potential of RNA modifiers in discovering novel therapeutics or immunotherapeutic procedures. Full article
(This article belongs to the Special Issue Autoimmune Blistering Diseases)
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