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      Juvenile pemphigus vulgaris: Literature review and a rare case report

      case-report

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          Key Clinical Message

          Pemphigus vulgaris (PV) is a chronic autoimmune blistering disorder characterized by the loss of intraepithelial adhesion affecting the skin and mucous membranes, predominantly affects females in their fifth and sixth decades of life. Due to its rare occurrence in children and adolescents, there is often a delay in diagnosis and treatment in this age group. PV should always be considered in the differential diagnosis of oral ulcerative and vesiculobullous lesions in both children and adolescents.

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          Pemphigus. S2 Guideline for diagnosis and treatment--guided by the European Dermatology Forum (EDF) in cooperation with the European Academy of Dermatology and Venereology (EADV).

          M. Hertl, H Jedlickova, S Karpati … (2015)
          Pemphigus encompasses a group of life-threatening autoimmune bullous diseases characterized by blisters and erosions of the mucous membranes and skin. Before the era of immunosuppressive treatment, the prognosis of pemphigus was almost fatal. Due to its rarity, only few prospective controlled therapeutic trials are available. For this reason, a group of European dermatologists with a long-standing interest and expertise in basic and clinical pemphigus research has sought to define diagnostic and therapeutic guidelines for the management of patients with pemphigus. This group identified the statements of major agreement or disagreement regarding the diagnostic and therapeutic management of pemphigus. The revised final version of the pemphigus guideline was finally passed on to the European Dermatology Forum (EDF) for a final consensus with the European Academy of Dermatology and Venereology (EADV) and the European Union of Medical Specialists (UEMS). © 2014 European Academy of Dermatology and Venereology.
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            Pemphigus: Current and Future Therapeutic Strategies

            Dario Didona, Roberto Maglie, Rüdiger Eming … (2019)
            Pemphigus encompasses a heterogeneous group of autoimmune blistering diseases, which affect both mucous membranes and the skin. The disease usually runs a chronic-relapsing course, with a potentially devastating impact on the patients' quality of life. Pemphigus pathogenesis is related to IgG autoantibodies targeting various adhesion molecules in the epidermis, including desmoglein (Dsg) 1 and 3, major components of desmosomes. The pathogenic relevance of such autoantibodies has been largely demonstrated experimentally. IgG autoantibody binding to Dsg results in loss of epidermal keratinocyte adhesion, a phenomenon referred to as acantholysis. This in turn causes intra-epidermal blistering and the clinical appearance of flaccid blisters and erosions at involved sites. Since the advent of glucocorticoids, the overall prognosis of pemphigus has largely improved. However, mortality persists elevated, since long-term use of high dose corticosteroids and adjuvant steroid-sparing immunosuppressants portend a high risk of serious adverse events, especially infections. Recently, rituximab, a chimeric anti CD20 monoclonal antibody which induces B-cell depletion, has been shown to improve patients' survival, as early rituximab use results in higher disease remission rates, long term clinical response and faster prednisone tapering compared to conventional immunosuppressive therapies, leading to its approval as a first line therapy in pemphigus. Other anti B-cell therapies targeting B-cell receptor or downstream molecules are currently tried in clinical studies. More intriguingly, a preliminary study in a preclinical mouse model of pemphigus has shown promise regarding future therapeutic application of Chimeric Autoantibody Receptor T-cells engineered using Dsg domains to selectively target autoreactive B-cells. Conversely, previous studies from our group have demonstrated that B-cell depletion in pemphigus resulted in secondary impairment of T-cell function; this may account for the observed long-term remission following B-cell recovery in rituximab treated patients. Likewise, our data support the critical role of Dsg-specific T-cell clones in orchestrating the inflammatory response and B-cell activation in pemphigus. Monitoring autoreactive T-cells in patients may indeed provide further information on the role of these cells, and would be the starting point for designating therapies aimed at restoring the lost immune tolerance against Dsg. The present review focuses on current advances, unmet challenges and future perspectives of pemphigus management.
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              Updated S2K guidelines on the management of pemphigus vulgaris and foliaceus initiated by the european academy of dermatology and venereology (EADV)

              P Joly, B Horváth, Α Patsatsi … (2020)
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                Author and article information

                Contributors
                Shamimul Hasan:
                ORCID: https://orcid.org/0000-0001-6832-9150
                shasan1@jmi.ac.in
                Journal
                Journal ID (nlm-ta): Clin Case Rep
                Journal ID (iso-abbrev): Clin Case Rep
                Journal ID (doi): 10.1002/(ISSN)2050-0904
                Journal ID (publisher-id): CCR3
                Title: Clinical Case Reports
                Publisher: John Wiley and Sons Inc. (Hoboken )
                ISSN (Electronic): 2050-0904
                Publication date (Electronic): 15 May 2024
                Publication date Collection: May 2024
                Volume: 12
                Issue: 5 ( doiID: 10.1002/ccr3.v12.5 )
                Electronic Location Identifier: e8954
                Affiliations
                [ 1 ] Department of Social and Public Health Ohio University Athens Ohio USA
                [ 2 ] Department of Oral Medicine and Radiology Faculty of Dentistry, Jamia Millia Islamia New Delhi India
                [ 3 ] Amity Institute of Public Health & Hospital Administration Amity University Noida Uttar Pradesh India
                [ 4 ] Oral and Maxillofacial Pathology Awadh Dental College and Hospital Jamshedpur Jharkhand India
                [ 5 ] Department of Oral Pathology and Microbiology, Saveetha Dental College and Hospitals, Saveetha Institute of Medical and Technical Sciences Saveetha University Chennai India
                Author notes
                [*] [* ] Correspondence

                Shamimul Hasan, Department of Oral Medicine and Radiology, Faculty of Dentistry, Jamia Millia Islamia, New Delhi, India.

                Email: shasan1@ 123456jmi.ac.in

                Author information
                https://orcid.org/0000-0001-6832-9150
                https://orcid.org/0000-0001-5213-296X
                https://orcid.org/0000-0003-1921-8336
                Article
                Publisher ID: CCR38954 Other ID: CCR3-2024-01-0263.R1
                DOI: 10.1002/ccr3.8954
                PMC ID: 11096281
                PubMed ID: 38756617
                SO-VID: 5bf331a1-b159-4a8a-b905-b28d1e6eac90
                Copyright © © 2024 The Author(s). Clinical Case Reports published by John Wiley & Sons Ltd.
                License:

                This is an open access article under the terms of the http://creativecommons.org/licenses/by/4.0/ License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.

                History
                Date revision received : 30 April 2024
                Date received : 29 January 2024
                Date accepted : 06 May 2024
                Page count
                Figures: 4, Tables: 1, Pages: 15, Words: 6300
                Categories
                Subject: Dentistry
                Subject: Dermatology
                Subject: Ear, Nose, and Throat
                Subject: Paediatrics and Adolescent Medicine
                Subject: Case Report
                Subject: Case Report
                Custom metadata
                source-schema-version-number 2.0
                cover-date May 2024
                details-of-publishers-convertor Converter:WILEY_ML3GV2_TO_JATSPMC version:6.4.3 mode:remove_FC converted:15.05.2024

                Keywords: autoimmune,histopathology,juvenile vulgaris,pemphigus,vesiculobullous disease
                Data availability:
                Keywords: autoimmune, histopathology, juvenile vulgaris, pemphigus, vesiculobullous disease

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