{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,30]],"date-time":"2026-10-30T23:52:05Z","timestamp":1793404325593,"version":"4.5.0"},"reference-count":118,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2025,3,3]],"date-time":"2025-03-03T00:00:00Z","timestamp":1740960000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Immunol."],"abstract":"<jats:p>Recently recognised as a desmosomal disorder, autoimmune pemphigus remains severe in some of its forms, such as pemphigus vulgaris. This review is divided into four parts. \u201cCellular and molecular mechanisms in autoimmune pemphigus\u201d discusses in detail antigenic targets, antibodies, immunological and genetic mechanisms of apoptosis and the involvement of cells and organelles (keratinocytes, lymphocytes, eosinophils and neutrophils) in different forms of pemphigus. These advances have led to today\u2019s first-line biologic therapy for pemphigus. The section \u201cSpecific features in the diagnosis of immune pemphigus\u201d deals with the clinical diagnostic clues (enanthema, intertrigo, pruritus, distribution of lesions). The third section, \u201cCharacteristics and challenges in different types of pemphigus\u201d, focuses on the importance of using standardised diagnostic criteria in paraneoplastic pemphigus and pemphigus herpetiformis, the specific and difficult situations of differentiation between bullous lupus and autoimmune Senear-Usher pemphigus, between IgA forms of pemphigus or differentiation with other autoimmune diseases or neutrophilic dermatoses. The possibility of subtype cross-reactivity in pemphigus is also discussed, as is the diagnosis and course of the disease in pregnant women. The final section is an update of the \u201cgold standard for the diagnosis and evaluation of autoimmune pemphigus\u201d, the role and place of direct immunofluorescence and additional serological tests. This revision is the first to combine the difficulties in clinical diagnosis with new molecular insights. It provides a comprehensive overview of recent advances in the understanding of autoimmune pemphigus, bridging the clinical challenges and complexities of diagnosing different forms of pemphigus, and is a valuable resource for clinicians caring for patients with pemphigus.<\/jats:p>","DOI":"10.3389\/fimmu.2025.1481093","type":"journal-article","created":{"date-parts":[[2025,3,3]],"date-time":"2025-03-03T05:16:42Z","timestamp":1740979002000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":13,"title":["Autoimmune pemphigus: difficulties in diagnosis and the molecular mechanisms underlying the disease"],"prefix":"10.3389","volume":"16","author":[{"given":"Olga","family":"Simionescu","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sorin Ioan","family":"Tudorache","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2025,3,3]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.3390\/antib11030055","article-title":"Mitochondrial autoantibodies and the role of apoptosis in pemphigus vulgaris","volume":"11","author":"Hutchison","year":"2022","journal-title":"Antibodies (Basel)."},{"key":"B2","doi-asserted-by":"publisher","first-page":"81","DOI":"10.1111\/joa.13634","article-title":"Structure and regulation of desmosomes in intercalated discs: Lessons from epithelia","volume":"242","author":"Yeruva","year":"2023","journal-title":"J Anat."},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2023.1259071","article-title":"Pemphigus vulgaris as an immune-related adverse event in recurrent metastatic esophageal squamous cell carcinoma treated with ipilimumab plus nivolumab: a case report and literature review","volume":"14","author":"Nakamura","year":"2023","journal-title":"Front Immunol"},{"key":"B4","doi-asserted-by":"publisher","first-page":"e2259","DOI":"10.1038\/cddis.2016.147","article-title":"ATP2C1 gene mutations in Hailey-Hailey disease and possible roles of SPCA1 isoforms in membrane trafficking","volume":"7","author":"Micaroni","year":"2016","journal-title":"Cell Death Dis"},{"key":"B5","doi-asserted-by":"publisher","first-page":"595","DOI":"10.1111\/bjd.20091","article-title":"Ca2+ signalling is critical for autoantibody-induced blistering of human epidermis in pemphigus","volume":"185","author":"Schmitt","year":"2021","journal-title":"Br J Dermatol"},{"key":"B6","doi-asserted-by":"publisher","first-page":"3","DOI":"10.1007\/978-3-540-68170-0_1","article-title":"Cadherin adhesion: mechanisms and molecular interactions","author":"Perez","year":"2004","journal-title":"Handb Exp Pharmacol"},{"key":"B7","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2022.884067","article-title":"Mechanisms causing acantholysis in pemphigus-lessons from human skin","volume":"13","author":"Egu","year":"2022","journal-title":"Front Immunol"},{"key":"B8","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1019003108","article-title":"Calcium-dependent dynamics of cadherin interactions at cell-cell junctions","volume":"108","author":"Kim","year":"2011","journal-title":"Proc Natl Acad Sci U S A."},{"key":"B9","doi-asserted-by":"publisher","DOI":"10.1111\/1346-8138.16660","article-title":"Significance of anti-desmocollin autoantibodies in pemphigus","volume":"50","author":"Ishii","year":"2023","journal-title":"J Dermatol"},{"key":"B10","doi-asserted-by":"publisher","DOI":"10.1016\/j.jdermsci.2007.05.005","article-title":"Three functions of cadherins in cell adhesion","volume":"23","author":"Ma\u00eetre","year":"2013","journal-title":"Curr Biol"},{"key":"B11","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1016\/j.jdermsci.2007.05.005.","article-title":"Beyond steric hindrance: the role of adhesion signaling pathways in the pathogenesis of pemphigus","volume":"48","author":"Sharma","year":"2007","journal-title":"J Dermatol Sci"},{"key":"B12","doi-asserted-by":"publisher","first-page":"2628","DOI":"10.1016\/jid.2024.03.042","article-title":"Desmosomal hyper-adhesion affects direct inhibition of desmoglein interactions in\u00a0pemphigus","volume":"144","author":"Steinert","year":"2024","journal-title":"J Invest Dermatol"},{"key":"B13","article-title":"Dsg1 and dsg3 composition of desmosomes across human epidermis and alterations in pemphigus vulgaris patient skin","volume":"25","author":"Schmitt","year":"2022","journal-title":"Front Immunol"},{"key":"B14","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2023.1163066","article-title":"Dsg3 epitope-specific signalling in pemphigus","volume":"14","author":"Schmitt","year":"2023","journal-title":"Front Immunol"},{"key":"B15","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2024.1346671","article-title":"Exploring the depths of IgG4: insights into autoimmunity and novel treatments","volume":"15","author":"\u00dcnl\u00fc","year":"2024","journal-title":"Front Immunol"},{"key":"B16","doi-asserted-by":"publisher","DOI":"10.3390\/antib12010004","article-title":"TLR7 and igM: dangerous partners in autoimmunity","volume":"12","author":"Amendt","year":"2023","journal-title":"Antibodies (Basel)."},{"key":"B17","doi-asserted-by":"publisher","DOI":"10.1093\/bjd\/ljae107","article-title":"Beyond the skin: B cells in pemphigus vulgaris, tolerance and treatment","volume":"191","author":"Strandmoe","year":"2024","journal-title":"Br J Dermatol"},{"key":"B18","doi-asserted-by":"publisher","DOI":"10.3390\/biomedicines9121795","article-title":"Soluble factors and receptors involved in skin innate immunity-what do we know so far","volume":"9","author":"Scurtu","year":"2021","journal-title":"Biomedicines."},{"key":"B19","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M607963200","article-title":"Anti-desmoglein 3 (Dsg3) monoclonal antibodies deplete desmosomes of Dsg3 and differ in their Dsg3-depleting activities related to pathogenicity","volume":"282","author":"Yamamoto","year":"2007","journal-title":"J Biol Chem"},{"key":"B20","doi-asserted-by":"publisher","DOI":"10.3390\/life12030329","article-title":"Marked to die-cell death mechanisms for keratinocyte acantholysis in pemphigus diseases","volume":"12","author":"Bumiller-Bini-Hoch","year":"2022","journal-title":"Life (Basel)."},{"key":"B21","doi-asserted-by":"publisher","DOI":"10.32607\/actanaturae.11890","article-title":"Smirnov IV. B cell profiling in patients with pemphigus vulgaris","volume":"15","author":"Abrikosova","year":"2023","journal-title":"Acta Naturae."},{"key":"B22","doi-asserted-by":"publisher","DOI":"10.1111\/cup.14395","article-title":"Lymphocyte-predominant lesional inflammatory infiltrates of the skin are associated with mucosal-dominant phenotype in pemphigus","volume":"50","author":"Papara","year":"2023","journal-title":"J Cutan Pathol"},{"key":"B23","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2020.102661","article-title":"The role of T cells in pemphigus vulgaris and bullous pemphigoid","volume":"19","author":"Fang","year":"2020","journal-title":"Autoimmun Rev"},{"key":"B24","doi-asserted-by":"publisher","DOI":"10.4049\/jimmunol.176.5.3215","article-title":"Inhibition of the transcription factor Foxp3 converts desmoglein 3-specific type 1 regulatory T cells into Th2-like cells","volume":"176","author":"Veldman","year":"2006","journal-title":"J Immunol"},{"key":"B25","doi-asserted-by":"publisher","DOI":"10.1016\/j.jid.2017.05.032","article-title":"Pivotal role of lesional and perilesional T\/B lymphocytes in pemphigus pathogenesis","volume":"137","author":"Yuan","year":"2017","journal-title":"J Invest Dermatol"},{"key":"B26","doi-asserted-by":"publisher","DOI":"10.1016\/j.imbio.2023.152340","article-title":"Deregulated phenotype of autoreactive Th17 and Treg clone cells in pemphigus vulgaris after in-vitro treatment with desmoglein antigen (Dsg-3)","volume":"228","author":"Ansari","year":"2023","journal-title":"Immunobiology."},{"key":"B27","doi-asserted-by":"publisher","DOI":"10.1016\/j.heliyon.2023.e16679","article-title":"Targeting therapy in pemphigus: Where are we now and where are we going","volume":"9","author":"Abulikemu","year":"2023","journal-title":"Heliyon."},{"key":"B28","article-title":"IgA pemphigus. 2024 may 1","volume-title":"StatPearls","author":"Aslanova","year":"2024"},{"key":"B29","doi-asserted-by":"publisher","DOI":"10.3389\/fmed.2018.00201","article-title":"The role of eosinophils in bullous pemphigoid: A developing model of eosinophil pathogenicity in mucocutaneous disease","volume":"5","author":"Amber","year":"2018","journal-title":"Front Med (Lausanne)."},{"key":"B30","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2017.01022","article-title":"Crosstalk between signaling pathways in pemphigus: A role for endoplasmic reticulum stress in p38 mitogen-activated protein kinase activation","volume":"8","author":"Cipolla","year":"2017","journal-title":"Front Immunol"},{"key":"B31","doi-asserted-by":"publisher","DOI":"10.1111\/odi.14480","article-title":"Diagnostic delay in autoimmune oral diseases","volume":"29","author":"Petruzzi","year":"2023","journal-title":"Oral Dis"},{"key":"B32","doi-asserted-by":"publisher","DOI":"10.1111\/jop.13052","article-title":"Clinical presentation and diagnostic delay in pemphigus vulgaris: A prospective study from Turkey","volume":"49","author":"Daltaban","year":"2020","journal-title":"J Oral Pathol Med"},{"key":"B33","doi-asserted-by":"publisher","DOI":"10.3390\/medicina57101080","article-title":"An updated review of pemphigus diseases","volume":"57","author":"Malik","year":"2021","journal-title":"Medicina (Kaunas)."},{"key":"B34","doi-asserted-by":"publisher","first-page":"52","DOI":"10.3390\/dermatopathology11010006","article-title":"Direct immunofluorescence of skin and oral mucosa: guidelines for selecting the optimum biopsy site","volume":"11","author":"Mahmood","year":"2024","journal-title":"Dermatopathology"},{"key":"B35","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2133.1981.tb08177.x","article-title":"Cerebriform tongue-a clinical sign in pemphigus vegetans","volume":"104","author":"Premalatha","year":"1981","journal-title":"Br J Dermatol"},{"key":"B36","doi-asserted-by":"publisher","DOI":"10.4103\/0019-5154.177760","article-title":"Cerebriform cutaneous lesions in pemphigus vegetans","volume":"61","author":"Rebello","year":"2016","journal-title":"Indian J Dermatol"},{"key":"B37","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2019.01259","article-title":"Paraneoplastic pemphigus: paraneoplastic autoimmune disease of the skin and mucosa","volume":"10","author":"Kim","year":"2019","journal-title":"Front Immunol"},{"key":"B38","article-title":"Pemphigus foliaceus. 2023 Aug 8","volume-title":"StatPearls","author":"Lepe","year":"2024"},{"key":"B39","doi-asserted-by":"publisher","DOI":"10.3390\/life11070621","article-title":"Evolving mechanisms in the pathophysiology of pemphigus vulgaris: A review emphasizing the role of desmoglein 3 in regulating p53 and the yes-associated protein","volume":"11","author":"Rehman","year":"2021","journal-title":"Life (Basel)."},{"key":"B40","doi-asserted-by":"publisher","DOI":"10.3390\/ijms241411493","article-title":"Plasma Exosomal microRNA Profile Reveals miRNA 148a-3p Downregulation in the Mucosal-Dominant Variant of Pemphigus Vulgaris","volume":"24","author":"Valentino","year":"2023","journal-title":"Int J Mol Sci"},{"key":"B41","article-title":"Paraneoplastic pemphigus. 2023 may 8","volume-title":"StatPearls","author":"Kappius","year":"2024"},{"key":"B42","doi-asserted-by":"publisher","DOI":"10.1590\/s1807-59322006000300016","article-title":"Pemphigus vegetans associated with verrucous lesions: expanding a phenotype","volume":"61","author":"de Almeida","year":"2006","journal-title":"Clinics (Sao Paulo)."},{"key":"B43","doi-asserted-by":"publisher","DOI":"10.5021\/ad.2011.23.S3.S310","article-title":"The neumann type of pemphigus vegetans treated with combination of dapsone and steroid","volume":"23","author":"Son","year":"2011","journal-title":"Ann Dermatol"},{"key":"B44","doi-asserted-by":"crossref","DOI":"10.1002\/9781119709268.rook050","article-title":"Immunobullous diseases","volume-title":"Rook\u2019s textbook of dermatology","author":"Schmidt","year":"2024"},{"key":"B45","doi-asserted-by":"publisher","DOI":"10.3390\/ijms18122532","article-title":"Paraneoplastic pemphigus: insight into the autoimmune pathogenesis, clinical features and therapy","volume":"18","author":"Paolino","year":"2017","journal-title":"Int J Mol Sci"},{"key":"B46","doi-asserted-by":"publisher","DOI":"10.1001\/archdermatol.2009.224","article-title":"Subcorneal pustular dermatosis-type IgA pemphigus with autoantibodies to desmocollins 1, 2, and 3","volume":"145","author":"D\u00fcker","year":"2009","journal-title":"Arch Dermatol"},{"key":"B47","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2019.01303","article-title":"Pruritus in autoimmune and inflammatory dermatoses","volume":"10","author":"Zeidler","year":"2019","journal-title":"Front Immunol"},{"key":"B48","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2020.01.025","article-title":"Prevalence and predictors of pruritus in pemphigus compared with bullous pemphigoid: A cross-sectional study","volume":"83","author":"Rolader","year":"2020","journal-title":"J Am Acad Dermatol"},{"key":"B49","doi-asserted-by":"publisher","DOI":"10.1111\/1346-8138.16730","article-title":"IL-31 and IL-31 receptor alpha in pemphigus: Contributors to more than just itch","volume":"50","author":"Okuno","year":"2023","journal-title":"J Dermatol"},{"key":"B50","article-title":"Pemphigus herpetiformis. 2023 jul 17","volume-title":"StatPearls","author":"Karray","year":"2024"},{"key":"B51","doi-asserted-by":"publisher","first-page":"997","DOI":"10.1111\/ijd.14395","article-title":"Clinical, pathologic, and immunologic features of pemphigus herpetiformis: a literature review and proposed diagnostic criteria","volume":"58","author":"Costa","year":"2019","journal-title":"Int J Dermatol"},{"key":"B52","doi-asserted-by":"publisher","DOI":"10.1016\/j.ad.2013.08.009","article-title":"Pemphigus herpetiformis with progression to pemphigus foliaceus: a case report","volume":"105","author":"Fuentes-Finkelstein","year":"2014","journal-title":"Actas Dermosifiliogr."},{"key":"B53","doi-asserted-by":"publisher","DOI":"10.1016\/j.abd.2022.09.012","article-title":"Is transition between subtypes of pemphigus possible? A series of pemphigus vulgaris patients showing the transition to pemphigus foliaceus","volume":"98","author":"Kucukoglu","year":"2023","journal-title":"Bras Dermatol"},{"key":"B54","doi-asserted-by":"publisher","DOI":"10.3349\/ymj.2006.47.2.278","article-title":"Transition from pemphigus foliaceus to pemphigus vulgaris: case report with literature review","volume":"47","author":"Park","year":"2006","journal-title":"Yonsei Med J"},{"key":"B55","article-title":"Bullous systemic lupus erythematosus. 2023 may 22","volume-title":"StatPearls","author":"Odonwodo","year":"2024"},{"key":"B56","doi-asserted-by":"publisher","first-page":"329","DOI":"10.4103\/0019-5154.114009","article-title":"A case of Senear-Usher syndrome","volume":"58","author":"Chavan","year":"2013","journal-title":"Indian J Dermatol"},{"key":"B57","doi-asserted-by":"publisher","DOI":"10.1155\/2012\/296214","article-title":"Autoantibodies in senear-usher syndrome: cross-reactivity or multiple autoimmunity","volume":"2012","author":"P\u00e9rez-P\u00e9rez","year":"2012","journal-title":"Autoimmune Dis"},{"key":"B58","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2024.1363032","article-title":"State-of-the-art diagnosis of autoimmune blistering diseases","volume":"15","author":"van Beek","year":"2024","journal-title":"Front Immunol"},{"key":"B59","doi-asserted-by":"publisher","DOI":"10.1111\/jdv.18419","article-title":"Vintage descriptions of IgA pemphigus","volume":"36","author":"Wallach","year":"2022","journal-title":"J Eur Acad Dermatol Venereol."},{"key":"B60","doi-asserted-by":"publisher","first-page":"790","DOI":"10.1007\/978-3-030-91557-5_11","article-title":"IgA pemphigus","author":"Horvath","year":"2022","journal-title":"Autoimmune Bullousd Diseases."},{"key":"B61","first-page":"295","volume-title":"Noninfectious vesiculobullous and vesiculopustular diseases in Lever\u2019s histopathology of the skin","author":"Wu","year":"2015"},{"key":"B62","article-title":"Detection of IgA\u00a0autoantibodies to desmogleins by an enzyme-linked immunosorbent assay: the presence of new minor subtypes of IgA pemphigus","volume":"137","author":"Hashimoto","year":"2001","journal-title":"Arch Dermatol"},{"key":"B63","doi-asserted-by":"publisher","DOI":"10.1016\/0190-9622(95)90402-6","article-title":"Atypical pemphigus associated with monoclonal IgA gammopathy","volume":"32","author":"Miyagawa","year":"1995","journal-title":"J Am Acad Dermatol"},{"key":"B64","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijwd.2018.02.002","article-title":"Autoimmune blistering disorders in the setting of human immunodeficiency virus infection","volume":"4","author":"Min","year":"2018","journal-title":"Int J Womens Dermatol"},{"key":"B65","doi-asserted-by":"publisher","DOI":"10.5217\/ir.2018.16.1.147","article-title":"A case of pemphigus vulgaris associated with ulcerative colitis","volume":"16","author":"Seo","year":"2018","journal-title":"Intest Res"},{"key":"B66","doi-asserted-by":"publisher","first-page":"3.815","DOI":"10.1016\/j.jaad.2022.06.726","article-title":"32677 IgA pemphigus in a patient with rheumatoid arthritis and cardiac amyloid successfully treated with oral dapsone","volume":"87","author":"Knabel","year":"2022","journal-title":"JAAD."},{"key":"B67","doi-asserted-by":"publisher","DOI":"10.1016\/j.jdcr.2024.02.025","article-title":"IgA pemphigus as an immune checkpoint inhibitor-associated skin manifestation","volume":"47","author":"Bruijn","year":"2024","journal-title":"JAAD Case Rep"},{"key":"B68","first-page":"1\u20136","article-title":"In xPharm: The Comprehensive Pharmacology Reference","author":"Sluzevich","year":"2007","journal-title":"Sci Direct."},{"key":"B69","doi-asserted-by":"publisher","DOI":"10.1111\/ijd.13025","article-title":"Clinicopathologic features of IgG\/IgA pemphigus in comparison with classic (IgG) and IgA pemphigus","volume":"55","author":"Toosi","year":"2016","journal-title":"Int J Dermatol"},{"key":"B70","doi-asserted-by":"publisher","DOI":"10.1016\/j.jdcr.2022.03.026","article-title":"IgG\/IgA pemphigus with differing regional presentations","volume":"28","author":"Cheng","year":"2022","journal-title":"JAAD Case Rep"},{"key":"B71","doi-asserted-by":"publisher","DOI":"10.23736\/S2784-8671.22.07217-6","article-title":"IgA pemphigus and Sneddon Wilkinson disease: a spectrum of diseases","volume":"157","author":"Aimo","year":"2022","journal-title":"Ital J Dermatol Venereol."},{"key":"B72","doi-asserted-by":"publisher","DOI":"10.1016\/j.det.2023.08.010","article-title":"Superficial and bullous neutrophilic dermatoses: sneddon-wilkin, igA pemphigus, and bullous lupus","volume":"42","author":"Manjaly","year":"2024","journal-title":"Dermatol Clin"},{"key":"B73","doi-asserted-by":"publisher","DOI":"10.11604\/pamj.2022.43.115.33116","article-title":"Maladie de Sneddon-Wilkinson: \u00e0 propos d\u2019un cas [Sneddon-Wilkinson disease: a case report","volume":"43","author":"Kerroum","year":"2022","journal-title":"Pan Afr Med J"},{"key":"B74","article-title":"Drug induced pemphigus. 2023 aug 7","volume-title":"StatPearls","author":"Pile","year":"2024"},{"key":"B75","doi-asserted-by":"publisher","DOI":"10.1016\/j.intimp.2020.107299","article-title":"Drug-induced pemphigus: A systematic review of 170 patients","volume":"92","author":"Ghaedi","year":"2021","journal-title":"Int Immunopharmacol."},{"key":"B76","doi-asserted-by":"publisher","DOI":"10.3389\/fmed.2023.1326359","article-title":"Pemphigus: trigger and predisposing factors","volume":"10","author":"Moro","year":"2023","journal-title":"Front Med (Lausanne)."},{"key":"B77","doi-asserted-by":"publisher","DOI":"10.1001\/jamadermatol.2022.1624","article-title":"Association of bullous pemphigoid with immune checkpoint inhibitor therapy in patients with cancer: A systematic review","volume":"158","author":"Asdourian","year":"2022","journal-title":"JAMA Dermatol"},{"key":"B78","doi-asserted-by":"publisher","DOI":"10.5681\/joddd.2012.008","article-title":"Pemphigus vulgaris associated with rheumatoid arthritis in a patient not taking penicillamine","volume":"6","author":"Gholizadeh","year":"2012","journal-title":"J Dent Res Dent Clin Dent Prospects."},{"key":"B79","doi-asserted-by":"publisher","DOI":"10.4103\/idoj.idoj_632_23","article-title":"Pemphigus and pregnancy","volume":"15","author":"De","year":"2024","journal-title":"Indian Dermatol Online J"},{"key":"B80","doi-asserted-by":"publisher","first-page":"99","DOI":"10.23736\/S2784-8671.23.07553-9","article-title":"Autoimmune bullous diseases in pregnancy: an overview of pathogenesis, clinical presentations, diagnostics and available therapies","volume":"158","author":"Vi\u010di\u0107","year":"2023","journal-title":"Ital J Dermatol Venerol."},{"key":"B81","doi-asserted-by":"publisher","DOI":"10.1111\/aji.13813","article-title":"Pregnancy in pemphigus vulgaris: A systematic review","volume":"91","author":"Lan","year":"2024","journal-title":"Am J Reprod Immunol"},{"key":"B82","doi-asserted-by":"publisher","DOI":"10.4067\/s0716-10182021000600800","article-title":"Reci\u00e9n nacido con p\u00e9nfigo sifil\u00edtico en tiempos de pandemia [Newborn with syphilitic pemphigus in pandemic\u2019s time","volume":"38","author":"Aguilante","year":"2021","journal-title":"Rev Chil Infectol."},{"key":"B83","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199012203232503","article-title":"Paraneoplastic pemphigus. An autoimmune mucocutaneous disease associated with neoplasia","volume":"323","author":"Anhalt","year":"1990","journal-title":"N Engl J Med"},{"key":"B84","doi-asserted-by":"publisher","first-page":"67","DOI":"10.1001\/archdermatol.2010.362","article-title":"Paraneoplastic pemphigus herpetiformis with IgG antibodies to desmoglein 3 and without mucosal lesions","volume":"147","author":"Prado","year":"2011","journal-title":"Arch Dermatol"},{"key":"B85","doi-asserted-by":"publisher","DOI":"10.1007\/s10238-024-01354-8","article-title":"Epidemiology of Malignant tumors in patients with pemphigus: an analysis of trends from 1955 to 2021","volume":"24","author":"Luo","year":"2024","journal-title":"Clin Exp Med"},{"key":"B86","doi-asserted-by":"publisher","DOI":"10.1136\/bcr-2023-258580","article-title":"Paraneoplastic pemphigus presenting as a prodrome to aggressive T cell lymphoma","volume":"17","author":"Bansari","year":"2024","journal-title":"BMJ Case Rep"},{"key":"B87","doi-asserted-by":"publisher","first-page":"16357","DOI":"10.1038\/s41598-020-73131-y","article-title":"Chronic lymphocytic leukemia-associated paraneoplastic pemphigus: potential cause and therapeutic strategies","volume":"10","author":"Cao","year":"2020","journal-title":"Sci Rep"},{"key":"B88","doi-asserted-by":"publisher","DOI":"10.7759\/cureus.36114","article-title":"Paraneoplastic pemphigus mimicking pemphigus vulgaris associated with castleman disease","volume":"15","author":"Grigore","year":"2023","journal-title":"Cureus."},{"key":"B89","doi-asserted-by":"publisher","DOI":"10.1111\/1346-8138.15289","article-title":"Paraneoplastic pemphigus associated with Waldenstr\u00f6m\u2019s macroglobulinemia","volume":"47","author":"Shirai","year":"2020","journal-title":"J Dermatol"},{"key":"B90","doi-asserted-by":"publisher","DOI":"10.1111\/j.1440-1843.2008.01397.x","article-title":"Constrictive bronchiolitis associated with paraneoplastic autoimmune multi-organ syndrome","volume":"14","author":"Maldonado","year":"2009","journal-title":"Respirology."},{"key":"B91","doi-asserted-by":"publisher","DOI":"10.1111\/jdv.18931","article-title":"S2k guidelines on the management of paraneoplastic pemphigus\/paraneoplastic autoimmune multiorgan syndrome initiated by the European Academy of Dermatology and Venereology (EADV)","volume":"37","author":"Antiga","year":"2023","journal-title":"J Eur Acad Dermatol Venereol."},{"key":"B92","doi-asserted-by":"publisher","DOI":"10.1111\/cup.14004","article-title":"Paraneoplastic pemphigus: Revised diagnostic criteria based on literature analysis","volume":"48","author":"Svoboda","year":"2021","journal-title":"J Cutan Pathol"},{"key":"B93","doi-asserted-by":"publisher","DOI":"10.1001\/archderm.138.1.95","article-title":"Clinical and serological transition from pemphigus vulgaris to pemphigus foliaceus demonstrated by desmoglein ELISA system","volume":"138","author":"Tsuji","year":"2002","journal-title":"Arch Dermatol"},{"key":"B94","doi-asserted-by":"publisher","DOI":"10.1016\/j.annder.2013.07.013","article-title":"Transition ph\u00e9notypique d\u2019un pemphigus vulgaire en pemphigus superficiel [Transition from pemphigus vulgaris to pemphigus foliaceus: a case report","volume":"140","author":"L\u00e9vy-Sitbon","year":"2013","journal-title":"Ann Dermatol Venereol."},{"key":"B95","doi-asserted-by":"publisher","DOI":"10.1684\/ejd.2014.2277","article-title":"Antibodies to the amino-terminal domain of desmoglein 1 are retained during transition from pemphigus vulgaris to pemphigus foliaceus","volume":"24","author":"Espa\u00f1a","year":"2014","journal-title":"Eur J Dermatol"},{"key":"B96","doi-asserted-by":"publisher","first-page":"49","DOI":"10.1007\/s40257-019-00477-z","article-title":"Hailey-hailey disease: an update review with a focus on treatment data","volume":"21","author":"Ben Lagha","year":"2020","journal-title":"Am J Clin Dermatol"},{"key":"B97","doi-asserted-by":"publisher","DOI":"10.3389\/fmed.2018.00226","article-title":"The genetics of pemphigus vulgaris","volume":"5","author":"Vodo","year":"2018","journal-title":"Front Med (Lausanne)."},{"key":"B98","doi-asserted-by":"publisher","DOI":"10.1590\/1678-4685-gmb-2019-0369","article-title":"Beyond the HLA polymorphism: A complex pattern of genetic susceptibility to pemphigus","volume":"43","author":"Petzl-Erler","year":"2020","journal-title":"Genet Mol Biol"},{"key":"B99","doi-asserted-by":"publisher","DOI":"10.1111\/bjd.19679","article-title":"ST18 affects cell-cell adhesion in pemphigus vulgaris in a tumour necrosis factor-\u03b1-dependent fashion","volume":"184","author":"Assaf","year":"2021","journal-title":"Br J Dermatol"},{"key":"B100","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pgen.1006008","article-title":"Identification of a functional risk variant for pemphigus vulgaris in the ST18 gene","volume":"12","author":"Vodo","year":"2016","journal-title":"PloS Genet"},{"key":"B101","doi-asserted-by":"publisher","first-page":"1","DOI":"10.4103\/2229-5178.198774","article-title":"Utility of immunofluorescence in dermatology","volume":"8","author":"Shetty","year":"2017","journal-title":"Indian Dermatol Online J"},{"key":"B102","doi-asserted-by":"publisher","DOI":"10.7759\/cureus.14562","article-title":"Utility of direct immunofluorescence in cutaneous autoimmune bullous disorders","volume":"13","author":"Brar","year":"2021","journal-title":"Cureus."},{"key":"B103","doi-asserted-by":"publisher","DOI":"10.4103\/2229-5178.156386","article-title":"Role of direct immunofluorescence in dermatological disorders","volume":"6","author":"Mysorekar","year":"2015","journal-title":"Indian Dermatol Online J"},{"key":"B104","doi-asserted-by":"publisher","first-page":"63","DOI":"10.4103\/2349-6029.195225","article-title":"The utility of immunofluorescence in diagnosing dermatological lesions and its correlation with clinical and histopathological diagnosis in a tertiary health care setup","volume":"3","author":"Dhanabalan","year":"2016","journal-title":"Indian J Dermatopathology Diagn Dermatol"},{"key":"B105","doi-asserted-by":"publisher","DOI":"10.1016\/j.clindermatol.2011.01.014","article-title":"IgA pemphigus.","volume":"29","author":"Tsuruta","year":"2011","journal-title":"Clin Dermatol"},{"key":"B106","doi-asserted-by":"publisher","first-page":"96","DOI":"10.1590\/abd1806-4841.20142459","article-title":"Non-classical forms of pemphigus: pemphigus herpetiformis, IgA pemphigus, paraneoplastic pemphigus and IgG\/IgA pemphigus","volume":"89","author":"Porro","year":"2014","journal-title":"Bras Dermatol"},{"key":"B107","doi-asserted-by":"publisher","DOI":"10.4103\/ijd.IJD_515_17","article-title":"Mitra P. A clinicopathological and immunofluorescence study of intraepidermal immunobullous diseases","volume":"64","author":"Basu","year":"2019","journal-title":"Indian J Dermatol"},{"key":"B108","doi-asserted-by":"publisher","DOI":"10.4103\/0019-5154.135488","article-title":"Gore CR. A cross-sectional study of direct immunofluorescence in the diagnosis of immunobullous dermatoses","volume":"59","author":"Buch","year":"2014","journal-title":"Indian J Dermatol"},{"key":"B109","doi-asserted-by":"publisher","DOI":"10.4103\/ijpm.ijpm_113_23","article-title":"Direct immunofluorescence (DIF) versus immunohistochemical (IHC) staining of complements and immunoglobulins (Ig) in pemphigus group","volume":"167","author":"Rana","year":"2024","journal-title":"Indian J Pathol Microbiol"},{"key":"B110","doi-asserted-by":"publisher","DOI":"10.1097\/DAD.0000000000000333","article-title":"Application of C4d immunohistochemistry on routinely processed tissue sections for the diagnosis of autoimmune bullous dermatoses","volume":"38","author":"Villani","year":"2016","journal-title":"Am J Dermatopathol."},{"key":"B111","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2019.01974","article-title":"Serological diagnosis of autoimmune bullous skin diseases","volume":"10","author":"Saschenbrecker","year":"2019","journal-title":"Front Immunol"},{"key":"B112","first-page":"79","article-title":"Comparison of diagnostic value of indirect immunofluorescence assay and desmoglein ELISA in the diagnosis of pemphigus","volume":"18","author":"Marinovi\u0107","year":"2010","journal-title":"Acta Dermatovenerol Croat."},{"key":"B113","doi-asserted-by":"publisher","DOI":"10.3389\/fmed.2018.00266","article-title":"The usefulness of indirect immunofluorescence in pemphigus and the natural history of patients with initial false-positive results: A retrospective cohort study","volume":"5","author":"Kridin","year":"2018","journal-title":"Front Med (Lausanne)."},{"key":"B114","doi-asserted-by":"publisher","DOI":"10.1007\/s00105-024-05428-2","article-title":"Hautfragilit\u00e4t bei blasenbildenden Autoimmundermatosen [Skin fragility in autoimmune blistering diseases of the skin","volume":"75","author":"Solimani","year":"2024","journal-title":"Dermatologie (Heidelb)."},{"key":"B115","doi-asserted-by":"publisher","DOI":"10.1111\/ijd.13824","article-title":"Antidesmoglein 1 and 3 antibodies in healthy subjects of a population in the Peruvian high amazon","volume":"57","author":"Ramos","year":"2018","journal-title":"Int J Dermatol"},{"key":"B116","doi-asserted-by":"publisher","DOI":"10.4103\/idoj.idoj_45_22","article-title":"Anti-desmoglein autoantibody in a patient with bullous pemphigoid - A case report","volume":"13","author":"Rai","year":"2022","journal-title":"Indian Dermatol Online J"},{"key":"B117","doi-asserted-by":"publisher","DOI":"10.1093\/cei\/uxad046","article-title":"SARS-CoV-2 infection is associated with anti-desmoglein 2 autoantibody detection","volume":"213","author":"Ward","year":"2023","journal-title":"Clin Exp Immunol"},{"key":"B118","doi-asserted-by":"publisher","DOI":"10.1016\/j.jdcr.2022.07.002","article-title":"New-onset pemphigus vegetans and pemphigus foliaceus after SARS-CoV-2 vaccination: A\u00a0report of 2 cases","author":"Gui","year":"2022","journal-title":"JAAD Case Rep"}],"container-title":["Frontiers in Immunology"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fimmu.2025.1481093\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,3,3]],"date-time":"2025-03-03T05:16:50Z","timestamp":1740979010000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fimmu.2025.1481093\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,3,3]]},"references-count":118,"alternative-id":["10.3389\/fimmu.2025.1481093"],"URL":"https:\/\/doi.org\/10.3389\/fimmu.2025.1481093","relation":{},"ISSN":["1664-3224"],"issn-type":[{"value":"1664-3224","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,3,3]]},"article-number":"1481093"}}