{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,12]],"date-time":"2026-02-12T13:06:16Z","timestamp":1770901576871,"version":"3.50.1"},"reference-count":122,"publisher":"MDPI AG","issue":"12","license":[{"start":{"date-parts":[[2022,6,19]],"date-time":"2022-06-19T00:00:00Z","timestamp":1655596800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Taiwan Ministry of Science and Technology","award":["MOST-109-2628-B-037-013"],"award-info":[{"award-number":["MOST-109-2628-B-037-013"]}]},{"name":"Taiwan Ministry of Science and Technology","award":["MOST-110-2628-B-037-007"],"award-info":[{"award-number":["MOST-110-2628-B-037-007"]}]},{"name":"Taiwan Ministry of Science and Technology","award":["MOST-110-2314-B-037-137"],"award-info":[{"award-number":["MOST-110-2314-B-037-137"]}]},{"name":"Taiwan Ministry of Science and Technology","award":["KMUH108-8T03"],"award-info":[{"award-number":["KMUH108-8T03"]}]},{"name":"Taiwan Ministry of Science and Technology","award":["KMUH109-9R67"],"award-info":[{"award-number":["KMUH109-9R67"]}]},{"name":"Taiwan Ministry of Science and Technology","award":["KMUH110-0R61"],"award-info":[{"award-number":["KMUH110-0R61"]}]},{"name":"Taiwan Ministry of Science and Technology","award":["KMU-TC109B03"],"award-info":[{"award-number":["KMU-TC109B03"]}]},{"name":"Taiwan Ministry of Science and Technology","award":["KMU-TC110B03"],"award-info":[{"award-number":["KMU-TC110B03"]}]},{"name":"Kaohsiung Medical University Hospital","award":["MOST-109-2628-B-037-013"],"award-info":[{"award-number":["MOST-109-2628-B-037-013"]}]},{"name":"Kaohsiung Medical University Hospital","award":["MOST-110-2628-B-037-007"],"award-info":[{"award-number":["MOST-110-2628-B-037-007"]}]},{"name":"Kaohsiung Medical University Hospital","award":["MOST-110-2314-B-037-137"],"award-info":[{"award-number":["MOST-110-2314-B-037-137"]}]},{"name":"Kaohsiung Medical University Hospital","award":["KMUH108-8T03"],"award-info":[{"award-number":["KMUH108-8T03"]}]},{"name":"Kaohsiung Medical University Hospital","award":["KMUH109-9R67"],"award-info":[{"award-number":["KMUH109-9R67"]}]},{"name":"Kaohsiung Medical University Hospital","award":["KMUH110-0R61"],"award-info":[{"award-number":["KMUH110-0R61"]}]},{"name":"Kaohsiung Medical University Hospital","award":["KMU-TC109B03"],"award-info":[{"award-number":["KMU-TC109B03"]}]},{"name":"Kaohsiung Medical University Hospital","award":["KMU-TC110B03"],"award-info":[{"award-number":["KMU-TC110B03"]}]},{"name":"Kaohsiung Medical University Research Center","award":["MOST-109-2628-B-037-013"],"award-info":[{"award-number":["MOST-109-2628-B-037-013"]}]},{"name":"Kaohsiung Medical University Research Center","award":["MOST-110-2628-B-037-007"],"award-info":[{"award-number":["MOST-110-2628-B-037-007"]}]},{"name":"Kaohsiung Medical University Research Center","award":["MOST-110-2314-B-037-137"],"award-info":[{"award-number":["MOST-110-2314-B-037-137"]}]},{"name":"Kaohsiung Medical University Research Center","award":["KMUH108-8T03"],"award-info":[{"award-number":["KMUH108-8T03"]}]},{"name":"Kaohsiung Medical University Research Center","award":["KMUH109-9R67"],"award-info":[{"award-number":["KMUH109-9R67"]}]},{"name":"Kaohsiung Medical University Research Center","award":["KMUH110-0R61"],"award-info":[{"award-number":["KMUH110-0R61"]}]},{"name":"Kaohsiung Medical University Research Center","award":["KMU-TC109B03"],"award-info":[{"award-number":["KMU-TC109B03"]}]},{"name":"Kaohsiung Medical University Research Center","award":["KMU-TC110B03"],"award-info":[{"award-number":["KMU-TC110B03"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Autoimmune bullous skin disorders are a group of disorders characterized by the formation of numerous blisters and erosions on the skin and\/or the mucosal membrane, arising from autoantibodies against the intercellular adhesion molecules and the structural proteins. They can be classified into intraepithelial or subepithelial autoimmune bullous dermatoses based on the location of the targeted antigens. These dermatoses are extremely debilitating and fatal in certain cases, depending on the degree of cutaneous and mucosal involvement. Effective treatments should be implemented promptly. Glucocorticoids serve as the first-line approach due to their rapid onset of therapeutic effects and remission of the acute phase. Nonetheless, long-term applications may lead to major adverse effects that outweigh the benefits. Hence, other adjuvant therapies are mandatory to minimize the potential harm and ameliorate the quality of life. Herein, we summarize the current therapeutic strategies and introduce promising therapies for intractable autoimmune bullous diseases.<\/jats:p>","DOI":"10.3390\/jcm11123528","type":"journal-article","created":{"date-parts":[[2022,6,19]],"date-time":"2022-06-19T10:14:52Z","timestamp":1655633692000},"page":"3528","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Current and Innovated Managements for Autoimmune Bullous Skin Disorders: An Overview"],"prefix":"10.3390","volume":"11","author":[{"given":"Kuan-Yu","family":"Chu","sequence":"first","affiliation":[{"name":"Department of Dermatology, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung 807378, Taiwan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hsin-Su","family":"Yu","sequence":"additional","affiliation":[{"name":"Graduate Institute of Clinical Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung 807378, Taiwan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2955-458X","authenticated-orcid":false,"given":"Sebastian","family":"Yu","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung 807378, Taiwan"},{"name":"Department of Dermatology, School of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung 807378, Taiwan"},{"name":"Neuroscience Research Center, Kaohsiung Medical University, Kaohsiung 807378, Taiwan"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2022,6,19]]},"reference":[{"key":"ref_1","first-page":"844","article-title":"Autoimmune bullous skin diseases. Part 1: Clinical manifestations","volume":"9","author":"Kneisel","year":"2011","journal-title":"J. Dtsch. Dermatol. Ges."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"465","DOI":"10.1024\/0040-5930\/a000080","article-title":"Autoimmune bullous skin disorders","volume":"67","author":"Hertl","year":"2010","journal-title":"Ther. Umschau. Rev. Ther."},{"key":"ref_3","first-page":"1077","article-title":"Diagnosis of autoimmune bullous diseases","volume":"16","author":"Zillikens","year":"2018","journal-title":"J. Dtsch. Dermatol. Ges. J. Ger. Soc. Dermatol. JDDG"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"1974","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":"ref_5","doi-asserted-by":"crossref","first-page":"482","DOI":"10.1016\/j.autrev.2014.01.047","article-title":"Diagnosis and classification of autoimmune blistering diseases","volume":"13","author":"Baum","year":"2014","journal-title":"Autoimmun. Rev."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"882","DOI":"10.1016\/S0140-6736(19)31778-7","article-title":"Pemphigus","volume":"394","author":"Schmidt","year":"2019","journal-title":"Lancet"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"16","DOI":"10.1111\/j.1365-4362.1985.tb05349.x","article-title":"Pemphigus erythematosus. Senear-Usher syndrome","volume":"24","author":"Amerian","year":"1985","journal-title":"Int. J. Dermatol."},{"key":"ref_8","first-page":"1159","article-title":"Subcorneal pustular dermatosis-type IgA pemphigus with autoantibodies to desmocollins 1, 2, and 3","volume":"145","author":"Schaller","year":"2009","journal-title":"Arch. Dermatol."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"1413","DOI":"10.3389\/fimmu.2019.01413","article-title":"Thymoma-Associated Paraneoplastic Autoimmune Multiorgan Syndrome\u2014From Pemphigus to Lichenoid Dermatitis","volume":"10","author":"Solimani","year":"2019","journal-title":"Front. Immunol."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"29","DOI":"10.1111\/j.1087-0024.2004.00832.x","article-title":"Paraneoplastic pemphigus","volume":"9","author":"Anhalt","year":"2004","journal-title":"J. Investig. Dermatol. Symp. Proc."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"553","DOI":"10.1016\/j.oraloncology.2003.09.020","article-title":"Neoplasms associated with paraneoplastic pemphigus: A review with emphasis on non-hematologic malignancy and oral mucosal manifestations","volume":"40","author":"Kaplan","year":"2004","journal-title":"Oral Oncol."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"1447","DOI":"10.1111\/bjd.14162","article-title":"Clinical and immunological findings in 104 cases of paraneoplastic pemphigus","volume":"173","author":"Ohzono","year":"2015","journal-title":"Br. J. Dermatol."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"296","DOI":"10.3389\/fmed.2018.00296","article-title":"Diagnosis of Autoimmune Blistering Diseases","volume":"5","author":"Witte","year":"2018","journal-title":"Front. Med."},{"key":"ref_14","doi-asserted-by":"crossref","unstructured":"Schmidt, E., della Torre, R., and Borradori, L. (2011). Clinical features and practical diagnosis of bullous pemphigoid. Dermatol. Clin., 29.","DOI":"10.1016\/j.det.2011.03.010"},{"key":"ref_15","doi-asserted-by":"crossref","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":"ref_16","doi-asserted-by":"crossref","first-page":"370","DOI":"10.1001\/archderm.138.3.370","article-title":"The first international consensus on mucous membrane pemphigoid: Definition, diagnostic criteria, pathogenic factors, medical treatment, and prognostic indicators","volume":"138","author":"Chan","year":"2002","journal-title":"Arch. Dermatol."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"45","DOI":"10.1016\/j.ophtha.2003.03.001","article-title":"Mucous membrane pemphigoid and pseudopemphigoid","volume":"111","author":"Thorne","year":"2004","journal-title":"Ophthalmology"},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"987","DOI":"10.1016\/0190-9622(91)70159-Y","article-title":"Cicatricial pemphigoid","volume":"24","author":"Ahmed","year":"1991","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"115","DOI":"10.1186\/s13023-019-1089-2","article-title":"Linear IgA bullous dermatosis in adults and children: A clinical and immunopathological study of 38 patients","volume":"14","author":"Genovese","year":"2019","journal-title":"Orphanet J. Rare Dis."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1001\/archderm.1971.04000130003001","article-title":"Epidermolysis bullosa acquisita. Report of three cases and review of all published cases","volume":"103","author":"Roenigk","year":"1971","journal-title":"Arch. Dermatol."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"2800","DOI":"10.1073\/pnas.0809230106","article-title":"Anti-laminin gamma-1 pemphigoid","volume":"106","author":"Dainichi","year":"2009","journal-title":"Proc. Natl. Acad. Sci USA"},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"2466","DOI":"10.3389\/fimmu.2019.02466","article-title":"Anti-p200 Pemphigoid: A Systematic Review","volume":"10","author":"Kridin","year":"2019","journal-title":"Front. Immunol."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"611","DOI":"10.25259\/IJDVL_886_19","article-title":"Biologics in autoimmune bullous diseases: Current scenario","volume":"87","author":"Bishnoi","year":"2021","journal-title":"Indian J. Dermatol. Venereol. Leprol."},{"key":"ref_24","first-page":"e2020050","article-title":"Pemphigus Vulgaris and Bullous Pemphigoid: Update on Diagnosis and Treatment","volume":"10","author":"Casanova","year":"2020","journal-title":"Dermatol. Pract. Concept."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"1127","DOI":"10.1046\/j.1365-2133.1999.02895.x","article-title":"The treatment of mild pemphigus vulgaris and pemphigus foliaceus with a topical corticosteroid","volume":"140","author":"Dumas","year":"1999","journal-title":"Br. J. Dermatol."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"203","DOI":"10.1001\/archderm.1996.03890260105016","article-title":"The adjuvant therapy of pemphigus: An update","volume":"132","author":"Bystryn","year":"1996","journal-title":"Arch. Dermatol."},{"key":"ref_27","doi-asserted-by":"crossref","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\u2014Brazilian Society of Dermatology","volume":"94","author":"Santi","year":"2019","journal-title":"An. Bras. Dermatol."},{"key":"ref_28","first-page":"833","article-title":"S2k guidelines for the treatment of pemphigus vulgaris\/foliaceus and bullous pemphigoid","volume":"13","author":"Eming","year":"2015","journal-title":"J. Dtsch. Dermatol. Ges. J. Ger. Soc. Dermatol. JDDG"},{"key":"ref_29","first-page":"329","article-title":"Pulse therapy and its modifications in pemphigus: A six year study","volume":"69","author":"Rao","year":"2003","journal-title":"Indian J. Dermatol. Venereol. Leprol."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"622","DOI":"10.1016\/j.jaad.2007.05.024","article-title":"Randomized controlled open-label trial of four treatment regimens for pemphigus vulgaris","volume":"57","author":"Esmaili","year":"2007","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"2031","DOI":"10.1016\/S0140-6736(17)30070-3","article-title":"First-line rituximab combined with short-term prednisone versus prednisone alone for the treatment of pemphigus (Ritux 3): A prospective, multicentre, parallel-group, open-label randomised trial","volume":"389","author":"Joly","year":"2017","journal-title":"Lancet"},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"746","DOI":"10.1016\/j.jaad.2015.11.008","article-title":"Future therapies for pemphigus vulgaris: Rituximab and beyond","volume":"74","author":"Huang","year":"2016","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"617","DOI":"10.1016\/j.jaad.2011.11.007","article-title":"Therapy with rituximab for autoimmune pemphigus: Results from a single-center observational study on 42 cases with long-term follow-up","volume":"67","author":"Cianchini","year":"2012","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"703","DOI":"10.1001\/jamadermatol.2013.6739","article-title":"Durable Remission of Pemphigus With a Fixed-Dose Rituximab Protocol","volume":"150","author":"Heelan","year":"2014","journal-title":"JAMA Dermatol."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"1341","DOI":"10.1111\/bjd.12972","article-title":"Clinical and immunological outcomes of high- and low-dose rituximab treatments in patients with pemphigus: A randomized, comparative, observer-blinded study","volume":"170","author":"Kanwar","year":"2014","journal-title":"Br. J. Dermatol."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"909","DOI":"10.1136\/ard.2010.144998","article-title":"Updated consensus statement on the use of rituximab in patients with rheumatoid arthritis","volume":"70","author":"Buch","year":"2011","journal-title":"Ann. Rheum. Dis."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"103","DOI":"10.4103\/ds.ds_7_21","article-title":"Venous thromboembolism in a case with pemphigus vulgaris after infusion of rituximab plus systemic glucocorticoids and azathioprine: A possible adverse effect of rituximab?","volume":"39","author":"Wu","year":"2021","journal-title":"Derm. Sin."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"337","DOI":"10.1016\/j.clindermatol.2009.06.018","article-title":"Adjuvant drugs in autoimmune bullous diseases, efficacy versus safety: Facts and controversies","volume":"28","author":"Schiavo","year":"2010","journal-title":"Clin. Derm."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"405","DOI":"10.1111\/jdv.12772","article-title":"Pemphigus. S2 Guideline for diagnosis and treatment\u2014Guided by the European Dermatology Forum (EDF) in cooperation with the European Academy of Dermatology and Venereology (EADV)","volume":"29","author":"Hertl","year":"2015","journal-title":"J. Eur. Acad. Dermatol. Venereol. JEADV"},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"78","DOI":"10.1016\/j.clindermatol.2011.03.013","article-title":"Immunosuppressive therapy for autoimmune bullous diseases","volume":"30","author":"Meurer","year":"2012","journal-title":"Clin. Derm."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"3440","DOI":"10.1172\/JCI24394","article-title":"Complete FcRn dependence for intravenous Ig therapy in autoimmune skin blistering diseases","volume":"115","author":"Li","year":"2005","journal-title":"J. Clin. Investig."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"1090","DOI":"10.3389\/fimmu.2019.01090","article-title":"High-Dose Intravenous Immunoglobulin in Skin Autoimmune Disease","volume":"10","author":"Hoffmann","year":"2019","journal-title":"Front. Immunol."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"600","DOI":"10.1016\/j.intimp.2005.11.002","article-title":"Comparison of cost of immune globulin intravenous therapy to conventional immunosuppressive therapy in treating patients with autoimmune mucocutaneous blistering diseases","volume":"6","author":"Daoud","year":"2006","journal-title":"Int. Immunopharmacol."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"315","DOI":"10.1016\/j.cyto.2007.12.007","article-title":"Cytokine profiles in pemphigus vulgaris patients treated with intravenous immunoglobulins as compared to conventional immunosuppressive therapy","volume":"41","author":"Keskin","year":"2008","journal-title":"Cytokine"},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"44","DOI":"10.1001\/archderm.1984.01650370050009","article-title":"Treatment of pemphigus vulgaris. Results obtained in 84 patients between 1961 and 1982","volume":"120","author":"Lever","year":"1984","journal-title":"Arch. Dermatol."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"429","DOI":"10.1016\/S0733-8635(18)30241-9","article-title":"Pemphigus vulgaris and vegetans","volume":"11","author":"Becker","year":"1993","journal-title":"Dermatol. Clin."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"382","DOI":"10.1016\/j.clindermatol.2013.01.005","article-title":"Pemphigus: Associations and management guidelines: Facts and controversies","volume":"31","author":"Ruocco","year":"2013","journal-title":"Clin. Derm."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"103","DOI":"10.1684\/ejd.2014.2483","article-title":"Pemphigus vulgaris: Approach to treatment","volume":"25","author":"Sinha","year":"2015","journal-title":"Eur. J. Dermatol. EJD"},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"154","DOI":"10.1111\/j.1365-2133.2011.10585.x","article-title":"Treatment of severe pemphigus with a combination of immunoadsorption, rituximab, pulsed dexamethasone and azathioprine\/mycophenolate mofetil: A pilot study of 23 patients","volume":"166","author":"Kasperkiewicz","year":"2012","journal-title":"Br. J. Dermatol."},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"881","DOI":"10.1111\/j.1610-0387.2007.06342.x","article-title":"Recommendations for the use of immunoapheresis in the treatment of autoimmune bullous diseases","volume":"5","author":"Zillikens","year":"2007","journal-title":"J. Dtsch. Dermatol. Ges. J. Ger. Soc. Dermatol. JDDG"},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"844","DOI":"10.1111\/j.1365-2133.2011.10732.x","article-title":"Combined treatment with immunoadsorption and rituximab leads to fast and prolonged clinical remission in difficult-to-treat pemphigus vulgaris","volume":"166","author":"Behzad","year":"2012","journal-title":"Br. J. Dermatol."},{"key":"ref_52","first-page":"13","article-title":"New anti-CD20 monoclonal antibodies for the treatment of B-cell lymphoid malignancies","volume":"25","author":"Robak","year":"2011","journal-title":"BioDrugs Clin. Immunother. Biopharm. Gene Ther."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"734","DOI":"10.1016\/j.jdcr.2020.05.034","article-title":"Refractory pemphigus vulgaris successfully treated with ofatumumab","volume":"6","author":"Klufas","year":"2020","journal-title":"JAAD Case Rep."},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"12","DOI":"10.1007\/s13317-017-0100-y","article-title":"Next-generation anti-CD20 monoclonal antibodies in autoimmune disease treatment","volume":"8","author":"Du","year":"2017","journal-title":"Auto-Immun. Highlights"},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"1331","DOI":"10.1001\/jamadermatol.2014.1939","article-title":"Subcutaneous veltuzumab, a humanized anti-CD20 antibody, in the treatment of refractory pemphigus vulgaris","volume":"150","author":"Ellebrecht","year":"2014","journal-title":"JAMA Derm."},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"179","DOI":"10.1126\/science.aaf6756","article-title":"Reengineering chimeric antigen receptor T cells for targeted therapy of autoimmune disease","volume":"353","author":"Ellebrecht","year":"2016","journal-title":"Science"},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"763","DOI":"10.1586\/1744666X.2016.1152888","article-title":"The role of Bruton\u2019s tyrosine kinase in autoimmunity and implications for therapy","volume":"12","author":"Crofford","year":"2016","journal-title":"Expert Rev. Clin. Immunol."},{"key":"ref_58","first-page":"67","article-title":"BTK Signaling in B Cell Differentiation and Autoimmunity","volume":"393","author":"Corneth","year":"2016","journal-title":"Curr. Top. Microbiol. Immunol."},{"key":"ref_59","doi-asserted-by":"crossref","unstructured":"Campbell, R., Chong, G., and Hawkes, E.A. (2018). Novel Indications for Bruton\u2019s Tyrosine Kinase Inhibitors, beyond Hematological Malignancies. J. Clin. Med., 7.","DOI":"10.3390\/jcm7040062"},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"1418","DOI":"10.3389\/fimmu.2019.01418","article-title":"Pemphigus: Current and Future Therapeutic Strategies","volume":"10","author":"Didona","year":"2019","journal-title":"Front. Immunol."},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"708071","DOI":"10.3389\/fmed.2021.708071","article-title":"Bruton Tyrosine Kinase Inhibition and Its Role as an Emerging Treatment in Pemphigus","volume":"8","author":"Patsatsi","year":"2021","journal-title":"Front. Med."},{"key":"ref_62","first-page":"e240","article-title":"Successful use of Bruton\u2019s kinase inhibitor, ibrutinib, to control paraneoplastic pemphigus in a patient with paraneoplastic autoimmune multiorgan syndrome and chronic lymphocytic leukaemia","volume":"58","author":"Lee","year":"2017","journal-title":"Australas J. Derm."},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"e447","DOI":"10.3324\/haematol.2017.168930","article-title":"The Bruton tyrosine kinase inhibitor CC-292 shows activity in mantle cell lymphoma and synergizes with lenalidomide and NIK inhibitors depending on nuclear factor-\u03baB mutational status","volume":"102","author":"Rodriguez","year":"2017","journal-title":"Haematologica"},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"745","DOI":"10.1111\/bjd.20431","article-title":"Proof of concept for the clinical effects of oral rilzabrutinib, the first Bruton tyrosine kinase inhibitor for pemphigus vulgaris: The phase II BELIEVE study","volume":"185","author":"Murrell","year":"2021","journal-title":"Br. J. Dermatol."},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"978","DOI":"10.3389\/fimmu.2019.00978","article-title":"Current Clinical Trials in Pemphigus and Pemphigoid","volume":"10","author":"Izumi","year":"2019","journal-title":"Front. Immunol."},{"key":"ref_66","doi-asserted-by":"crossref","first-page":"465","DOI":"10.1038\/nri844","article-title":"BAFF: A fundamental survival factor for B cells","volume":"2","author":"Mackay","year":"2002","journal-title":"Nat. Rev. Immunol."},{"key":"ref_67","doi-asserted-by":"crossref","first-page":"441","DOI":"10.1016\/S1074-7613(04)00079-2","article-title":"Reduced competitiveness of autoantigen-engaged B cells due to increased dependence on BAFF","volume":"20","author":"Lesley","year":"2004","journal-title":"Immunity"},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"87","DOI":"10.1111\/j.1600-0625.2006.00485.x","article-title":"Elevated serum BAFF levels in patients with localized scleroderma in contrast to other organ-specific autoimmune diseases","volume":"16","author":"Matsushita","year":"2007","journal-title":"Exp. Derm."},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"59","DOI":"10.1172\/JCI0214121","article-title":"Association of BAFF\/BLyS overexpression and altered B cell differentiation with Sj\u00f6gren\u2019s syndrome","volume":"109","author":"Groom","year":"2002","journal-title":"J. Clin. Investig."},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"1313","DOI":"10.1002\/1529-0131(200106)44:6<1313::AID-ART223>3.0.CO;2-S","article-title":"Elevated serum B lymphocyte stimulator levels in patients with systemic immune-based rheumatic diseases","volume":"44","author":"Cheema","year":"2001","journal-title":"Arthritis Rheum."},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"777","DOI":"10.1007\/s10875-010-9468-4","article-title":"Neonatal Fc receptor: From immunity to therapeutics","volume":"30","author":"Kuo","year":"2010","journal-title":"J. Clin. Immunol."},{"key":"ref_72","doi-asserted-by":"crossref","first-page":"e2661","DOI":"10.1212\/WNL.0000000000007600","article-title":"Randomized phase 2 study of FcRn antagonist efgartigimod in generalized myasthenia gravis","volume":"92","author":"Howard","year":"2019","journal-title":"Neurology"},{"key":"ref_73","doi-asserted-by":"crossref","first-page":"178","DOI":"10.1002\/ajh.25680","article-title":"Phase 2 study of efgartigimod, a novel FcRn antagonist, in adult patients with primary immune thrombocytopenia","volume":"95","author":"Newland","year":"2020","journal-title":"Am. J. Hematol."},{"key":"ref_74","doi-asserted-by":"crossref","first-page":"4372","DOI":"10.1172\/JCI97911","article-title":"Neonatal Fc receptor antagonist efgartigimod safely and sustainably reduces IgGs in humans","volume":"128","author":"Ulrichts","year":"2018","journal-title":"J. Clin. Investig."},{"key":"ref_75","doi-asserted-by":"crossref","first-page":"293","DOI":"10.1111\/exd.12919","article-title":"Induction of T regulatory cells by the superagonistic anti-CD28 antibody D665 leads to decreased pathogenic IgG autoantibodies against desmoglein 3 in a HLA-transgenic mouse model of pemphigus vulgaris","volume":"25","author":"Schmidt","year":"2016","journal-title":"Exp. Derm."},{"key":"ref_76","doi-asserted-by":"crossref","first-page":"1960","DOI":"10.1681\/ASN.2016111206","article-title":"Cell Therapy in Kidney Transplantation: Focus on Regulatory T Cells","volume":"28","author":"Zwang","year":"2017","journal-title":"J. Am. Soc. Nephrol."},{"key":"ref_77","doi-asserted-by":"crossref","first-page":"107","DOI":"10.4103\/ds.ds_9_21","article-title":"An elderly female with pemphigus foliaceus possibly induced by losartan\/hydrochlorothiazide","volume":"39","author":"Wang","year":"2021","journal-title":"Derm. Sin."},{"key":"ref_78","doi-asserted-by":"crossref","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":"ref_79","first-page":"1339","article-title":"Bullous autoimmune dermatoses","volume":"16","author":"Hofmann","year":"2018","journal-title":"J. Dtsch. Dermatol. Ges. J. Ger. Soc. Dermatol. JDDG"},{"key":"ref_80","doi-asserted-by":"crossref","first-page":"47","DOI":"10.4103\/ds.ds_33_20","article-title":"Intraepidermal neutrophilic dermatosis-type immunoglobulin A pemphigus","volume":"39","author":"Hong","year":"2021","journal-title":"Derm. Sin."},{"key":"ref_81","doi-asserted-by":"crossref","first-page":"923","DOI":"10.1067\/mjd.2000.104002","article-title":"Rapid response of IgA pemphigus of subcorneal pustular dermatosis type to treatment with isotretinoin","volume":"43","author":"Gruss","year":"2000","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_82","doi-asserted-by":"crossref","first-page":"1040","DOI":"10.1046\/j.1365-2133.2002.504815.x","article-title":"A case of IgA pemphigus successfully treated with acitretin","volume":"147","author":"Hashimoto","year":"2002","journal-title":"Br. J. Dermatol."},{"key":"ref_83","doi-asserted-by":"crossref","first-page":"541","DOI":"10.1016\/j.jaad.2005.02.051","article-title":"Rapid response of IgA pemphigus of the subcorneal pustular dermatosis subtype to treatment with adalimumab and mycophenolate mofetil","volume":"53","author":"Howell","year":"2005","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_84","doi-asserted-by":"crossref","first-page":"162","DOI":"10.1111\/j.1365-4632.2009.03995.x","article-title":"Paraneoplastic pemphigus\/paraneoplastic autoimmune multiorgan syndrome","volume":"48","author":"Sehgal","year":"2009","journal-title":"Int. J. Dermatol."},{"key":"ref_85","doi-asserted-by":"crossref","first-page":"142","DOI":"10.1002\/1096-8652(200102)66:2<142::AID-AJH1032>3.0.CO;2-0","article-title":"Successful treatment of paraneoplastic pemphigus in follicular NHL with rituximab: Report of a case and review of treatment for paraneoplastic pemphigus in NHL and CLL","volume":"66","author":"Heizmann","year":"2001","journal-title":"Am. J. Hematol."},{"key":"ref_86","doi-asserted-by":"crossref","first-page":"571","DOI":"10.1007\/s00011-017-1032-8","article-title":"The immunological function of CD52 and its targeting in organ transplantation","volume":"66","author":"Zhao","year":"2017","journal-title":"Inflamm. Res."},{"key":"ref_87","doi-asserted-by":"crossref","first-page":"16414","DOI":"10.3390\/ijms160716414","article-title":"Alemtuzumab in Multiple Sclerosis: Mechanism of Action and Beyond","volume":"16","author":"Ruck","year":"2015","journal-title":"Int. J. Mol. Sci."},{"key":"ref_88","doi-asserted-by":"crossref","unstructured":"Paolino, G., Didona, D., Magliulo, G., Iannella, G., Didona, B., Mercuri, S.R., Moliterni, E., Donati, M., Ciofalo, A., and Granata, G. (2017). Paraneoplastic Pemphigus: Insight into the Autoimmune Pathogenesis, Clinical Features and Therapy. Int. J. Mol. Sci., 18.","DOI":"10.3390\/ijms18122532"},{"key":"ref_89","doi-asserted-by":"crossref","first-page":"206","DOI":"10.1111\/j.1600-0609.2004.00280.x","article-title":"Alemtuzumab-induced remission of both severe paraneoplastic pemphigus and leukaemic bone marrow infiltration in a case of treatment-resistant B-cell chronic lymphocytic leukaemia","volume":"73","author":"Hohwy","year":"2004","journal-title":"Eur. J. Haematol."},{"key":"ref_90","doi-asserted-by":"crossref","first-page":"469","DOI":"10.1111\/bjd.12324","article-title":"Alemtuzumab is effective against severe chronic lymphocytic leukaemia-associated paraneoplastic pemphigus","volume":"169","author":"Bech","year":"2013","journal-title":"Br. J. Dermatol."},{"key":"ref_91","doi-asserted-by":"crossref","first-page":"321","DOI":"10.1056\/NEJMoa011592","article-title":"A comparison of oral and topical corticosteroids in patients with bullous pemphigoid","volume":"346","author":"Joly","year":"2002","journal-title":"N. Engl. J. Med."},{"key":"ref_92","doi-asserted-by":"crossref","first-page":"1681","DOI":"10.1038\/jid.2008.412","article-title":"A comparison of two regimens of topical corticosteroids in the treatment of patients with bullous pemphigoid: A multicenter randomized study","volume":"129","author":"Joly","year":"2009","journal-title":"J. Invest. Derm."},{"key":"ref_93","first-page":"Cd002292","article-title":"Interventions for bullous pemphigoid","volume":"2010","author":"Kirtschig","year":"2010","journal-title":"Cochrane Database Syst. Rev."},{"key":"ref_94","doi-asserted-by":"crossref","first-page":"613","DOI":"10.1016\/j.det.2011.06.003","article-title":"Evidence-based management of bullous pemphigoid","volume":"29","author":"Daniel","year":"2011","journal-title":"Dermatol. Clin."},{"key":"ref_95","doi-asserted-by":"crossref","first-page":"80","DOI":"10.1002\/cia2.12126","article-title":"Doxycycline as an initial treatment of bullous pemphigoid in Japanese patients","volume":"3","author":"Kimura","year":"2020","journal-title":"J. Cutan. Immunol. Allergy"},{"key":"ref_96","doi-asserted-by":"crossref","first-page":"30","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":"Huebner","year":"2017","journal-title":"JAMA Dermatol."},{"key":"ref_97","doi-asserted-by":"crossref","first-page":"468","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":"ref_98","doi-asserted-by":"crossref","first-page":"1778","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. JEADV"},{"key":"ref_99","doi-asserted-by":"crossref","first-page":"e469","DOI":"10.1111\/jdv.17229","article-title":"Omalizumab: An underutilized treatment option in bullous pemphigoid patients with co-morbidities","volume":"35","author":"De","year":"2021","journal-title":"J. Eur. Acad. Dermatol. Venereol. JEADV"},{"key":"ref_100","doi-asserted-by":"crossref","first-page":"15003","DOI":"10.1074\/jbc.M111.221036","article-title":"The C5a receptor on mast cells is critical for the autoimmune skin-blistering disease bullous pemphigoid","volume":"286","author":"Heimbach","year":"2011","journal-title":"J. Biol. Chem."},{"key":"ref_101","doi-asserted-by":"crossref","first-page":"18001","DOI":"10.1038\/srep18001","article-title":"Integrating longitudinal serum IL-17 and IL-23 follow-up, along with autoantibodies variation, contributes to predict bullous pemphigoid outcome","volume":"5","author":"Giustiniani","year":"2015","journal-title":"Sci. Rep."},{"key":"ref_102","doi-asserted-by":"crossref","first-page":"112","DOI":"10.1046\/j.1365-2133.2000.03599.x","article-title":"Elevated levels of eotaxin and interleukin-5 in blister fluid of bullous pemphigoid: Correlation with tissue eosinophilia","volume":"143","author":"Wakugawa","year":"2000","journal-title":"Br. J. Dermatol."},{"key":"ref_103","doi-asserted-by":"crossref","first-page":"1079","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":"ref_104","doi-asserted-by":"crossref","first-page":"1141","DOI":"10.1046\/j.1365-2133.2003.05265.x","article-title":"The role of T lymphocytes and cytokines in the pathogenesis of pemphigoid gestationis","volume":"148","author":"Fabbri","year":"2003","journal-title":"Br. J. Dermatol."},{"key":"ref_105","doi-asserted-by":"crossref","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":"ref_106","doi-asserted-by":"crossref","first-page":"34","DOI":"10.1016\/j.coph.2018.12.007","article-title":"Pharmacological advances in pemphigoid","volume":"46","author":"Maglie","year":"2019","journal-title":"Curr. Opin. Pharm."},{"key":"ref_107","doi-asserted-by":"crossref","first-page":"669","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":"ref_108","doi-asserted-by":"crossref","first-page":"846","DOI":"10.1002\/jlb.65.6.846","article-title":"Depletion of eosinophils in mice through the use of antibodies specific for C-C chemokine receptor 3 (CCR3)","volume":"65","author":"Grimaldi","year":"1999","journal-title":"J. Leukoc. Biol."},{"key":"ref_109","doi-asserted-by":"crossref","first-page":"161","DOI":"10.1016\/j.oooo.2015.01.024","article-title":"World Workshop on Oral Medicine VI: A systematic review of the treatment of mucous membrane pemphigoid","volume":"120","author":"Taylor","year":"2015","journal-title":"Oral Surg. Oral Med. Oral Pathol. Oral Radiol."},{"key":"ref_110","doi-asserted-by":"crossref","first-page":"604","DOI":"10.2340\/00015555-1142","article-title":"Oral mucous membrane pemphigoid: Complete response to topical tacrolimus","volume":"91","author":"Lee","year":"2011","journal-title":"Acta Derm. Venereol."},{"key":"ref_111","doi-asserted-by":"crossref","first-page":"1102","DOI":"10.1111\/1346-8138.15111","article-title":"Japanese guidelines for the management of pemphigoid (including epidermolysis bullosa acquisita)","volume":"46","author":"Ujiie","year":"2019","journal-title":"J. Derm."},{"key":"ref_112","doi-asserted-by":"crossref","first-page":"652","DOI":"10.1016\/j.jaad.2005.11.1102","article-title":"Treatment of linear IgA bullous dermatosis of childhood with flucloxacillin","volume":"54","author":"Alajlan","year":"2006","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_113","doi-asserted-by":"crossref","first-page":"1259","DOI":"10.1517\/14656566.2011.549127","article-title":"Current concepts in the treatment of epidermolysis bullosa acquisita","volume":"12","author":"Ahmed","year":"2011","journal-title":"Expert Opin. Pharm."},{"key":"ref_114","doi-asserted-by":"crossref","first-page":"321","DOI":"10.1016\/j.jdcr.2015.07.007","article-title":"Mycophenolate mofetil (MMF) in the treatment of epidermolysis bullosa acquisita (EBA) long-term follow-up","volume":"1","author":"Sami","year":"2015","journal-title":"JAAD Case Rep."},{"key":"ref_115","doi-asserted-by":"crossref","first-page":"827","DOI":"10.1016\/S0190-9622(09)80064-9","article-title":"Epidermolysis bullosa acquisita: Efficacy of high-dose intravenous immunoglobulins","volume":"31","author":"Caldwell","year":"1994","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_116","doi-asserted-by":"crossref","first-page":"417","DOI":"10.1111\/j.1365-2133.2007.08048.x","article-title":"Treatment-resistant classical epidermolysis bullosa acquisita responding to rituximab","volume":"157","author":"Sadler","year":"2007","journal-title":"Br. J. Dermatol."},{"key":"ref_117","doi-asserted-by":"crossref","first-page":"146","DOI":"10.1006\/clim.2001.5113","article-title":"Treatment of epidermolysis bullosa acquisita with the humanized anti-Tac mAb daclizumab","volume":"101","author":"Egan","year":"2001","journal-title":"Clin. Immunol."},{"key":"ref_118","first-page":"523","article-title":"Dermatitis herpetiformis: Problems, progress and prospects","volume":"12","author":"Fry","year":"2002","journal-title":"Eur. J. Dermatol. EJD"},{"key":"ref_119","doi-asserted-by":"crossref","first-page":"759","DOI":"10.1111\/j.1365-2133.1989.tb08218.x","article-title":"Sulphamethoxypyridazine for dermatitis herpetiformis, linear IgA disease and cicatricial pemphigoid","volume":"121","author":"McFadden","year":"1989","journal-title":"Br. J. Dermatol."},{"key":"ref_120","doi-asserted-by":"crossref","first-page":"101","DOI":"10.1111\/j.1440-0960.2005.00152.x","article-title":"Sulfasalazine and dermatitis herpetiformis","volume":"46","author":"Willsteed","year":"2005","journal-title":"Australas J. Derm."},{"key":"ref_121","doi-asserted-by":"crossref","first-page":"1306","DOI":"10.1001\/archdermatol.2011.303","article-title":"IgA Anti-p200 Pemphigoid","volume":"147","author":"Wozniak","year":"2011","journal-title":"Arch. Dermatol."},{"key":"ref_122","doi-asserted-by":"crossref","first-page":"185","DOI":"10.1016\/j.jaad.2014.02.036","article-title":"Anti-p200 pemphigoid","volume":"71","author":"Goletz","year":"2014","journal-title":"J. Am. Acad. Dermatol."}],"container-title":["Journal of Clinical Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2077-0383\/11\/12\/3528\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T23:35:14Z","timestamp":1760139314000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2077-0383\/11\/12\/3528"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,6,19]]},"references-count":122,"journal-issue":{"issue":"12","published-online":{"date-parts":[[2022,6]]}},"alternative-id":["jcm11123528"],"URL":"https:\/\/doi.org\/10.3390\/jcm11123528","relation":{},"ISSN":["2077-0383"],"issn-type":[{"value":"2077-0383","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,6,19]]}}}