{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,14]],"date-time":"2026-10-14T16:16:20Z","timestamp":1791994580579,"version":"4.4.0"},"reference-count":53,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2023,7,20]],"date-time":"2023-07-20T00:00:00Z","timestamp":1689811200000},"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>Bullous pemphigoid (BP) is a common subepidermal bullous disorder that lacks adequate treatment alternatives. Dupilumab, an anti-interleukin (IL) 4 receptor \u03b1 antibody blocking Th2 molecules IL-4 and 13, has been used off-label and shown to be effective in refractory BP cases.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>BP patients with various disease severities and comorbidities were included in this case series. All patients received dupilumab alone or in combination with immunosuppressants in a real-world setting. Complete remission (CR) was defined as the absence of pruritus symptoms and previous BP eruptions, with only hyperpigmentation patches and without newly occurring lesions for at least 4 weeks. Disease relapse was classified as the appearance of three or more new lesions within 1 month or at least one large urticarial or eczematous lesion that did not resolve within a week.<\/jats:p><\/jats:sec><jats:sec><jats:title>Findings<\/jats:title><jats:p>Ten individuals were enrolled in this case series. Pruritus symptoms and BP eruptions improved significantly in nine patients (90%). Seven patients (70%) attained CR, including all mild-to-moderate (100%) cases and three of six (50%) severe BP cases. At the dupilumab monotherapy stage, eosinophilia was observed in two severe cases. One patient out of seven (14.3%) relapsed after 1 year of follow-up after CR.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Treatment of BP with diverse comorbidities with anti-IL-4 receptor \u03b1 antibody provides further credentials to a prospective randomized study. More impressive efficacy and safety profiles were observed in patients with mild-to-moderate disease after 1 year of follow-up. Eosinophilia may occur in patients receiving dupilumab monotherapy.<\/jats:p><\/jats:sec>","DOI":"10.3389\/fimmu.2023.1165106","type":"journal-article","created":{"date-parts":[[2023,7,21]],"date-time":"2023-07-21T02:27:24Z","timestamp":1689906444000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":14,"title":["Anti-interleukin 4 receptor \u03b1 antibody for the treatment of Chinese bullous pemphigoid patients with diverse comorbidities and a 1-year follow-up: a monocentric real-world study"],"prefix":"10.3389","volume":"14","author":[{"given":"Si-Hang","family":"Wang","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ying","family":"Shan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Si-Zhe","family":"Li","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ya-Gang","family":"Zuo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2023,7,20]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.1111\/bjd.12072","article-title":"British Association of Dermatologists' guidelines for the management of bullous pemphigoid 2012","volume":"167","author":"Venning","year":"2012","journal-title":"Br J Dermatol"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.1007\/s40257-018-0401-6","article-title":"Rituximab and Omalizumab for the treatment of bullous pemphigoid: a systematic review of the literature","volume":"20","author":"Kremer","year":"2019","journal-title":"Am J Clin Dermatol"},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.1111\/jdv.13758","article-title":"Successful treatment of bullous pemphigoid with omalizumab as corticosteroid-sparing agent: report of two cases and review of literature","volume":"30","author":"Balakirski","year":"2016","journal-title":"J Eur Acad Dermatol Venereol"},{"key":"B4","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2020.03.017","article-title":"Interleukin 4 and interleukin 13 inhibition: A promising therapeutic approach in bullous pemphigoid","volume":"83","author":"Geller","year":"2020","journal-title":"J Am Acad Dermatol"},{"key":"B5","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2014.10.013","article-title":"Dupilumab improves the molecular signature in skin of patients with moderate-to-severe atopic dermatitis","volume":"134","author":"Hamilton","year":"2014","journal-title":"J Allergy Clin Immunol"},{"key":"B6","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2021.738907","article-title":"Efficacy and safety of dupilumab in moderate-to-severe bullous pemphigoid","volume":"12","author":"Zhang","year":"2021","journal-title":"Front Immunol"},{"key":"B7","doi-asserted-by":"publisher","DOI":"10.1016\/j.annder.2021.08.005","article-title":"French Study Group on Autoimmune Bullous Skin: Updated French guidelines for the therapeutic management of bullous pemphigoid","volume":"149","author":"Castel","year":"2021","journal-title":"Ann Dermatol Venereol"},{"key":"B8","doi-asserted-by":"publisher","DOI":"10.1111\/bjd.19611","article-title":"International validation of the Bullous Pemphigoid Disease Area Index severity score and calculation of cut-off values for defining mild, moderate and severe types of bullous pemphigoid","volume":"184","author":"Masmoudi","year":"2021","journal-title":"Br J Dermatol"},{"key":"B9","doi-asserted-by":"publisher","DOI":"10.1007\/s11684-018-0649-5","article-title":"Immunoregulatory effects of Tripterygium wilfordii Hook F and its extracts in clinical practice","volume":"13","author":"Luo","year":"2019","journal-title":"Front Med"},{"key":"B10","doi-asserted-by":"publisher","DOI":"10.1111\/dth.13721","article-title":"Treatment of bullous pemphigoid in Chinese patients with Tripterygium wilfordii Hook F","volume":"33","author":"Ge","year":"2020","journal-title":"Dermatol Ther"},{"key":"B11","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaut.2016.06.019","article-title":"IgG4 autoantibodies are inhibitory in the autoimmune disease bullous pemphigoid","volume":"73","author":"Zuo","year":"2016","journal-title":"J Autoimmun"},{"key":"B12","doi-asserted-by":"publisher","DOI":"10.1001\/jamadermatol.2016.3357","article-title":"Correlation of serum levels of IgE autoantibodies against BP180 with bullous pemphigoid disease activity","volume":"153","author":"van Beek","year":"2017","journal-title":"JAMA Dermatol"},{"key":"B13","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":"B14","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2133.1999.02907.x","article-title":"Characterization of skin cytokines in bullous pemphigoid and pemphigus vulgaris","volume":"140","author":"Rico","year":"1999","journal-title":"Br J Dermatol"},{"key":"B15","doi-asserted-by":"publisher","DOI":"10.1046\/j.0022-202x.2001.01505.x","article-title":"Skin-homing interleukin-4 and -13-producing cells contribute to bullous pemphigoid: remission of disease is associated with increased frequency of interleukin-10-producing cells","volume":"117","author":"Teraki","year":"2001","journal-title":"J Invest Dermatol"},{"key":"B16","doi-asserted-by":"publisher","DOI":"10.1016\/s0923-1811(02)00067-1","article-title":"Th1, Th2 and Th3 cytokines in the pathogenesis of bullous pemphigoid","volume":"30","author":"Giomi","year":"2002","journal-title":"J Dermatol Sci"},{"key":"B17","doi-asserted-by":"publisher","DOI":"10.18176\/jiaci.0682","article-title":"Dupilumab: a review of present indications and uses out of indication","volume":"32","author":"Munoz-Bellido","year":"2021","journal-title":"J Investig Allergol Clin Immunol"},{"key":"B18","doi-asserted-by":"publisher","first-page":"46","DOI":"10.1016\/j.jaad.2020.01.089","article-title":"Dupilumab as a novel therapy for bullous pemphigoid: a multicenter case series","volume":"83","author":"Abdat","year":"2020","journal-title":"J Am Acad Dermatol"},{"key":"B19","doi-asserted-by":"publisher","DOI":"10.1016\/j.jdcr.2022.09.017","article-title":"Recalcitrant bullous pemphigoid responsive to dupilumab in an adolescent patient","volume":"29","author":"Zhou","year":"2022","journal-title":"JAAD Case Rep"},{"key":"B20","doi-asserted-by":"publisher","DOI":"10.1111\/jdv.18673","article-title":"Severe bullous pemphigoid following Covid-19 vaccination resistant to rituximab and successfully treated with dupilumab","volume":"37","author":"Baffa","year":"2022","journal-title":"J Eur Acad Dermatol Venereol"},{"key":"B21","doi-asserted-by":"publisher","DOI":"10.1111\/dth.15890","article-title":"A severe case of IgA bullous pemphigoid successfully treated with dupilumab","volume":"35","author":"Valenti","year":"2022","journal-title":"Dermatol Ther"},{"key":"B22","doi-asserted-by":"publisher","DOI":"10.1111\/dth.15727","article-title":"Severe pemphigoid nodularis successfully treated with dupilumab","volume":"35","author":"Jendoubi","year":"2022","journal-title":"Dermatol Ther"},{"key":"B23","doi-asserted-by":"publisher","DOI":"10.1111\/ijd.16451","article-title":"Successful treatment of eczema-like bullous pemphigoid and typical bullous pemphigoid with dupilumab: two case reports","volume":"62","author":"Xu","year":"2022","journal-title":"Int J Dermatol"},{"key":"B24","doi-asserted-by":"publisher","DOI":"10.5070\/D328257396","article-title":"A case of nivolumab-induced bullous pemphigoid successfully treated with dupilumab","volume":"28","author":"Bruni","year":"2022","journal-title":"Dermatol Online J"},{"key":"B25","doi-asserted-by":"publisher","DOI":"10.1111\/dth.15623","article-title":"Dupilumab for the treatment of pembrolizumab-induced bullous pemphigoid: A case report","volume":"35","author":"Pop","year":"2022","journal-title":"Dermatol Ther"},{"key":"B26","doi-asserted-by":"publisher","DOI":"10.1111\/dth.15243","article-title":"The treatment of refractory atypical bullous pemphigoid with generalized eczema and intense pruritus with dupilumab","volume":"35","author":"Li","year":"2022","journal-title":"Dermatol Ther"},{"key":"B27","doi-asserted-by":"publisher","DOI":"10.1111\/dth.15330","article-title":"A successful case of vesicular pemphigoid concurrent with pulmonary tuberculosis with dupilumab","volume":"35","author":"Shan","year":"2022","journal-title":"Dermatol Ther"},{"key":"B28","doi-asserted-by":"publisher","DOI":"10.1111\/jdv.17999","article-title":"Efficacy and safety of methotrexate, omalizumab and dupilumab for bullous pemphigoid in patients resistant or contraindicated to oral steroids","volume":"36","author":"Velin","year":"2022","journal-title":"A monocentric real-life study. J Eur Acad Dermatol Venereol"},{"key":"B29","doi-asserted-by":"publisher","DOI":"10.1111\/dth.15541","article-title":"Case Report: Dupilumab for the treatment of bullous pemphigoid","volume":"35","author":"Wang","year":"2022","journal-title":"Dermatol Ther"},{"key":"B30","doi-asserted-by":"publisher","DOI":"10.1111\/1346-8138.16428","article-title":"Treatment of bullous pemphigoid with dupilumab: Dupilumab exerts its effect by primarily suppressing T-helper 2 cytokines","volume":"49","author":"Takamura","year":"2022","journal-title":"J Dermatol"},{"key":"B31","doi-asserted-by":"publisher","DOI":"10.1111\/dth.15648","article-title":"Dupilumab combined with low-dose systemic steroid therapy improves efficacy and safety for bullous pemphigoid","volume":"35","author":"Yang","year":"2022","journal-title":"Dermatol Ther"},{"key":"B32","doi-asserted-by":"publisher","DOI":"10.1007\/s13555-022-00851-6","article-title":"The combination of dupilumab with other monoclonal antibodies","volume":"13","author":"Gisondi","year":"2022","journal-title":"Dermatol Ther (Heidelb)"},{"key":"B33","doi-asserted-by":"publisher","DOI":"10.1111\/bjd.19890","article-title":"Mortality and clinical response of patients with bullous pemphigoid treated with rituximab","volume":"185","author":"Yoo","year":"2021","journal-title":"Br J Dermatol"},{"key":"B34","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa2205982","article-title":"Dupilumab in adults and adolescents with eosinophilic esophagitis","volume":"387","author":"Dellon","year":"2022","journal-title":"N Engl J Med"},{"key":"B35","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1804092","article-title":"Dupilumab efficacy and safety in moderate-to-severe uncontrolled asthma","volume":"378","author":"Castro","year":"2018","journal-title":"N Engl J Med"},{"key":"B36","doi-asserted-by":"publisher","DOI":"10.1111\/jdv.17177","article-title":"Dupilumab-associated hypereosinophilia in patients treated for moderate-to-severe atopic dermatitis","volume":"35","author":"Marcant","year":"2021","journal-title":"J Eur Acad Dermatol Venereol"},{"key":"B37","doi-asserted-by":"publisher","DOI":"10.1111\/ddg.12612","article-title":"S2k guideline for the diagnosis of pemphigus vulgaris\/foliaceus and bullous pemphigoid","volume":"13","author":"Schmidt","year":"2015","journal-title":"J Dtsch Dermatol Ges"},{"key":"B38","doi-asserted-by":"publisher","first-page":"700","DOI":"10.1016\/j.jaad.2015.11.030","article-title":"Treatment of recalcitrant bullous pemphigoid (BP) with a novel protocol: A retrospective study with a 6-year follow-up","volume":"74","author":"Ahmed","year":"2016","journal-title":"J Am Acad Dermatol"},{"key":"B39","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2018.00248","article-title":"Effectiveness and safety of rituximab in recalcitrant pemphigoid diseases","volume":"9","author":"Lamberts","year":"2018","journal-title":"Front Immunol"},{"key":"B40","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2019.03.049","article-title":"Rituximab therapy in patients with bullous pemphigoid: A retrospective study of 20 patients","volume":"81","author":"Polansky","year":"2019","journal-title":"J Am Acad Dermatol"},{"key":"B41","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2019.11.023","article-title":"Clinical outcome and safety of rituximab therapy for pemphigoid diseases","volume":"82","author":"Tovanabutra","year":"2020","journal-title":"J Am Acad Dermatol"},{"key":"B42","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2008.11.035","article-title":"Pathogenicity of IgE in autoimmunity: successful treatment of bullous pemphigoid with Omalizumab","volume":"123","author":"Fairley","year":"2009","journal-title":"J Allergy Clin Immunol"},{"key":"B43","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2133.2011.10748.x","article-title":"Successful management of severe infant bullous pemphigoid with omalizumab","volume":"166","author":"Dufour","year":"2012","journal-title":"Br J Dermatol"},{"key":"B44","doi-asserted-by":"publisher","DOI":"10.1001\/archdermatol.2012.1604","article-title":"Successful treatment of bullous pemphigoid with omalizumab","volume":"148","author":"London","year":"2012","journal-title":"Arch Dermatol"},{"key":"B45","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2014.04.053","article-title":"Omalizumab therapy for bullous pemphigoid","volume":"71","author":"Yu","year":"2014","journal-title":"J Am Acad Dermatol"},{"key":"B46","doi-asserted-by":"publisher","DOI":"10.4103\/0378-6323.183628","article-title":"Bullous pemphigoid successfully treated with omalizumab","volume":"82","author":"Gonul","year":"2016","journal-title":"Indian J Dermatol Venereol Leprol"},{"key":"B47","doi-asserted-by":"publisher","first-page":"33","DOI":"10.1590\/abd1806-4841.2019940207","article-title":"Consensus on the treatment of autoimmune bullous dermatoses: bullous pemphigoid, mucous membrane pemphigoid and epidermolysis bullosa acquisita - Brazilian Society of Dermatology","volume":"94","author":"Santi","year":"2019","journal-title":"Bras Dermatol"},{"key":"B48","doi-asserted-by":"publisher","DOI":"10.2340\/00015555-2845","article-title":"Biological and clinical response to omalizumab in a patient with bullous pemphigoid","volume":"98","author":"Menzinger","year":"2018","journal-title":"Acta Derm Venereol"},{"key":"B49","doi-asserted-by":"publisher","DOI":"10.1001\/jamadermatol.2022.1156","article-title":"Evaluation of nomacopan for treatment of bullous pemphigoid: A phase 2a nonrandomized controlled trial","volume":"158","author":"Sadik","year":"2022","journal-title":"JAMA Dermatol"},{"key":"B50","doi-asserted-by":"publisher","DOI":"10.1111\/all.13950","article-title":"Mepolizumab failed to affect bullous pemphigoid: A randomized, placebo-controlled, double-blind phase 2 pilot study","volume":"75","author":"Simon","year":"2020","journal-title":"Allergy"},{"key":"B51","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":"B52","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaut.2018.09.003","article-title":"IL-17A is functionally relevant and a potential therapeutic target in bullous pemphigoid","volume":"96","author":"Chakievska","year":"2019","journal-title":"J Autoimmun"},{"key":"B53","doi-asserted-by":"publisher","DOI":"10.1111\/dth.13958","article-title":"Total IgE, eosinophils, and interleukins 16, 17A, and 23 correlations in severe bullous pemphigoid and treatment implications","volume":"33","author":"Delli","year":"2020","journal-title":"Dermatol Ther"}],"container-title":["Frontiers in Immunology"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fimmu.2023.1165106\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,7,21]],"date-time":"2023-07-21T02:27:27Z","timestamp":1689906447000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fimmu.2023.1165106\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,7,20]]},"references-count":53,"alternative-id":["10.3389\/fimmu.2023.1165106"],"URL":"https:\/\/doi.org\/10.3389\/fimmu.2023.1165106","relation":{},"ISSN":["1664-3224"],"issn-type":[{"value":"1664-3224","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,7,20]]},"article-number":"1165106"}}