{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,2]],"date-time":"2026-07-02T15:09:16Z","timestamp":1783004956987,"version":"3.54.5"},"reference-count":23,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2022,9,8]],"date-time":"2022-09-08T00:00:00Z","timestamp":1662595200000},"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:sec><jats:title>Background<\/jats:title><jats:p>Pemphigus is a group of autoimmune blistering diseases including Pemphigus vulgaris (PV) and Pemphigus foliaceus (PF). These conditions exhibit lesions with mucosal or mucocutaneous (PV) or cutaneous (PF) morphology, as framed by the Desmoglein Compensation Hypothesis (DCH). However, some PV patients present with solely cutaneous disease (cPV), and growing evidence suggests the existence of a cPV subtype <jats:italic>without any history<\/jats:italic> of mucosal erosions\/blisters (cPVwohm), neither of which are predicted by the DCH.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Participants were recruited from several outpatient clinical settings and patient support group meetings throughout the US. On intake, subjects provided blood samples and completed questionnaires regarding their disease status.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>We report three cases of clinically and histologically confirmed cPV without history of mucosal lesions (cPVwohm). Of these patients, two do not carry the most common PV associated HLA alleles, DRB1*0402 or DQB1*0503. The same two patients also tested negative for the primary PV associated autoantibodies, anti-desmoglein 3 and anti-desmoglein 1, while in active disease status.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>We confirm the first documented individual cases of cPVwohm in North America, supporting the existence of PV patients that develop cutaneous disease without a history of mucosal lesions, challenging the fidelity of the DCH. Two of the 3 patients reported did not type for the common PV-associated HLA genes or display anti-desmoglein autoantibodies while in active disease, suggesting cPV patients may develop Pemphigus <jats:italic>via<\/jats:italic> genetic and immune mechanisms that differ from typical mucosal or mucocutaneous PV.<\/jats:p><\/jats:sec>","DOI":"10.3389\/fimmu.2022.969279","type":"journal-article","created":{"date-parts":[[2022,9,8]],"date-time":"2022-09-08T07:00:23Z","timestamp":1662620423000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":5,"title":["Case report: Documentation of cutaneous only pemphigus vulgaris without history of mucosal lesions in North America"],"prefix":"10.3389","volume":"13","author":[{"given":"John","family":"Baker","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kristina","family":"Seiffert-Sinha","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Animesh A.","family":"Sinha","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2022,9,8]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.1016\/S0190-9622(99)70183-0","article-title":"The clinical phenotype of pemphigus is defined by the anti-desmoglein autoantibody profile","volume":"40","author":"Amagai","year":"1999","journal-title":"J Am Acad Dermatol"},{"key":"B2","doi-asserted-by":"publisher","first-page":"17026","DOI":"10.1038\/nrdp.2017.26","article-title":"Pemphigus","volume":"3","author":"Kasperkiewicz","year":"2017","journal-title":"Nat Rev Dis Primers"},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.1159\/000088513","article-title":"Pemphigus vulgaris with no mucosal lesions showing pemphigus-foliaceus-like skin manifestations: Is there a \u2018cutaneous type\u2019 of pemphigus vulgaris","volume":"211","author":"Nagasaka","year":"2005","journal-title":"Dermatology"},{"key":"B4","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2005.01.106","article-title":"Cutaneous type pemphigus vulgaris: A rare clinical phenotype of pemphigus","volume":"52","author":"Yoshida","year":"2005","journal-title":"J Am Acad Dermatol"},{"key":"B5","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2230.2008.02871.x","article-title":"Cutaneous pemphigus vulgaris with skin features similar to the classic mucocutaneous type: A case report and review of the literature","volume":"33","author":"Shinkuma","year":"2008","journal-title":"Clin Exp Dermatol"},{"key":"B6","doi-asserted-by":"publisher","DOI":"10.1111\/ajd.12154","article-title":"Cutaneous pemphigus vulgaris with absence of desmoglein 1 autoantibodies. an example of the extended desmoglein compensation theory","volume":"55","author":"Carew","year":"2014","journal-title":"Australas J Dermatol"},{"key":"B7","doi-asserted-by":"publisher","DOI":"10.1046\/j.0022-202x.2001.01703.x","article-title":"Unusual pemphigus phenotype in the presence of a Dsg1 and Dsg3 autoantibody profile","volume":"118","author":"M\u00fcller","year":"2002","journal-title":"J Invest Dermatol"},{"key":"B8","doi-asserted-by":"publisher","first-page":"86","DOI":"10.2340\/00015555-1854","article-title":"Development of a disease registry for autoimmune bullous diseases: Initial analysis of the pemphigus vulgaris subset","volume":"95","author":"Shah","year":"2015","journal-title":"Acta Derm. 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