{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,22]],"date-time":"2026-10-22T22:27:09Z","timestamp":1792708029522,"version":"4.5.0"},"reference-count":52,"publisher":"MDPI AG","issue":"10","license":[{"start":{"date-parts":[[2021,10,4]],"date-time":"2021-10-04T00:00:00Z","timestamp":1633305600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Medicina"],"abstract":"<jats:p>The pemphigoid family of dermatoses is characterized by autoimmune subepidermal blistering. The classic paradigm for pemphigoid, and the most common member, is bullous pemphigoid. Its variable clinical presentation, with or without frank bullae, is linked by significant pruritus afflicting the elderly. Mucous membrane pemphigoid is an umbrella term for a group of subepidermal blistering dermatoses that favor the mucosal membranes and can scar. Epidermolysis bullosa acquisita is a chronic blistering disorder characterized by skin fragility, sensitivity to trauma, and its treatment-refractory nature. Clinicians that encounter these pemphigoid disorders may benefit from an overview of their clinical presentation, diagnostic work-up, and therapeutic management, with an emphasis on the most frequently encountered pemphigoid disease, bullous pemphigoid.<\/jats:p>","DOI":"10.3390\/medicina57101061","type":"journal-article","created":{"date-parts":[[2021,10,11]],"date-time":"2021-10-11T01:57:54Z","timestamp":1633917474000},"page":"1061","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":38,"title":["Bullous Pemphigoid and Other Pemphigoid Dermatoses"],"prefix":"10.3390","volume":"57","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3886-1626","authenticated-orcid":false,"given":"Valeryia","family":"Pratasava","sequence":"first","affiliation":[{"name":"College of Medicine, Drexel University, Philadelphia, PA 19129, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vikram N.","family":"Sahni","sequence":"additional","affiliation":[{"name":"College of Medicine, Drexel University, Philadelphia, PA 19129, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5937-6083","authenticated-orcid":false,"given":"Aishwarya","family":"Suresh","sequence":"additional","affiliation":[{"name":"College of Medicine, Drexel University, Philadelphia, PA 19129, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3318-0143","authenticated-orcid":false,"given":"Simo","family":"Huang","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Lewis Katz School of Medicine, Temple University, Philadelphia, PA 19140, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Abhirup","family":"Are","sequence":"additional","affiliation":[{"name":"College of Medicine, University of Florida, Gainesville, FL 32606, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sylvia","family":"Hsu","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Lewis Katz School of Medicine, Temple University, Philadelphia, PA 19140, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0562-0826","authenticated-orcid":false,"given":"Kiran","family":"Motaparthi","sequence":"additional","affiliation":[{"name":"Department of Dermatology, College of Medicine, University of Florida, Gainesville, FL 32606, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2021,10,4]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"a180","DOI":"10.1136\/bmj.a180","article-title":"Bullous pemphigoid and pemphigus vulgaris--incidence and mortality in the UK: Population based cohort study","volume":"337","author":"Langan","year":"2008","journal-title":"BMJ"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"220","DOI":"10.3389\/fmed.2018.00220","article-title":"The Growing Incidence of Bullous Pemphigoid: Overview and Potential Explanations","volume":"5","author":"Kridin","year":"2018","journal-title":"Front. Med."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"133","DOI":"10.1590\/abd1806-4841.20199007","article-title":"Bullous pemphigoid","volume":"94","author":"Miyamoto","year":"2019","journal-title":"An. Bras. Dermatol."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"26","DOI":"10.1007\/s12016-017-8633-4","article-title":"Autoimmune Subepidermal Bullous Diseases of the Skin and Mucosae: Clinical Features, Diagnosis, and Management","volume":"54","author":"Amber","year":"2017","journal-title":"Clin. Rev. Allergy Immunol."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"611","DOI":"10.1016\/j.cden.2013.07.003","article-title":"Mucous Membrane Pemphigoid","volume":"57","author":"Xu","year":"2013","journal-title":"Dent. Clin. N. Am."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"520","DOI":"10.1111\/j.1600-0625.2007.00564.x","article-title":"Experimental models of epidermolysis bullosa acquisita","volume":"16","author":"Sitaru","year":"2007","journal-title":"Exp. Dermatol."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"48","DOI":"10.1001\/archderm.1995.01690130050009","article-title":"Incidence and distribution of subepidermal autoimmune bullous skin diseases in three French regions. Bullous Diseases French Study Group","volume":"131","author":"Bernard","year":"1995","journal-title":"Arch. Dermatol."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"115","DOI":"10.2340\/00015555-0819","article-title":"Serological Studies in Bullous Pemphigoid: A Literature Review of Antibody Titers at Presentation and in Clinical Remission","volume":"90","author":"Leuci","year":"2010","journal-title":"Acta Derm. Venereol."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"445","DOI":"10.1016\/j.autrev.2017.03.010","article-title":"Bullous Pemphigoid","volume":"16","author":"Ruzicka","year":"2017","journal-title":"Autoimmun. Rev."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"784","DOI":"10.1046\/j.1523-1747.2003.12146.x","article-title":"Identification of a Potential Effector Function for IgE Autoantibodies in the Organ-Specific Autoimmune Disease Bullous Pemphigoid","volume":"120","author":"Dimson","year":"2003","journal-title":"J. Investig. Dermatol."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"18","DOI":"10.1016\/j.jim.2009.04.013","article-title":"A novel ELISA reveals high frequencies of BP180-specific IgE production in bullous pemphigoid","volume":"346","author":"Messingham","year":"2009","journal-title":"J. Immunol. Methods"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"1506","DOI":"10.3389\/fimmu.2019.01506","article-title":"New Insights Into the Pathogenesis of Bullous Pemphigoid: 2019 Update","volume":"10","author":"Genovese","year":"2019","journal-title":"Front. Immunol."},{"key":"ref_13","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_14","doi-asserted-by":"crossref","first-page":"59","DOI":"10.1177\/1203475420943260","article-title":"Cutaneous Immune-Related Adverse Events (irAEs) to Immune Checkpoint Inhibitors: A Dermatology Perspective on Management","volume":"25","author":"Muntyanu","year":"2020","journal-title":"J. Cutan. Med. Surg."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1016\/j.clindermatol.2011.03.005","article-title":"Bullous pemphigoid: From the clinic to the bench","volume":"30","author":"Zambruno","year":"2012","journal-title":"Clin. Dermatol."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"257","DOI":"10.1111\/j.1365-2133.1993.tb11843.x","article-title":"Anti-HLA antibodies in pemphigoid gestationis (herpes gestationis)","volume":"129","author":"Shornick","year":"1993","journal-title":"Br. J. Dermatol."},{"key":"ref_17","doi-asserted-by":"crossref","unstructured":"Ludwig, R.J., and Zillikens, D. (2015). Pathogenesis of Epidermolysis Bullosa Acquisita. Blistering Diseases, Springer.","DOI":"10.1007\/978-3-662-45698-9_12"},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"1111","DOI":"10.1111\/j.1365-2133.2012.11108.x","article-title":"Clinical presentation and diagnostic delay in bullous pemphigoid: A prospective nationwide cohort","volume":"167","author":"Combescure","year":"2012","journal-title":"Br. J. Dermatol."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"430","DOI":"10.1111\/bjd.20068","article-title":"Differences in the clinical presentation of bullous pemphigoid in patients with skin of colour and patients with white skin","volume":"185","author":"Shah","year":"2021","journal-title":"Br. J. Dermatol."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"438","DOI":"10.1016\/j.autrev.2015.01.006","article-title":"Atypical presentations of bullous pemphigoid: Clinical and immunopathological aspects","volume":"14","author":"Cozzani","year":"2015","journal-title":"Autoimmun. Rev."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"185","DOI":"10.1186\/s13023-014-0185-6","article-title":"Bullous pemphigoid in infants: Characteristics, diagnosis and treatment","volume":"9","author":"Moellmann","year":"2014","journal-title":"Orphanet J. Rare Dis."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1016\/j.jaad.2007.08.010","article-title":"Bullous pemphigoid in infancy: Clinical and epidemiologic characteristics","volume":"58","author":"Cohen","year":"2008","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"7","DOI":"10.1111\/bjd.16440","article-title":"The International Bullous Diseases Group consensus on diagnostic criteria for epidermolysis bullosa acquisita: A useful tool for dermatologists","volume":"179","author":"Yamagami","year":"2018","journal-title":"Br. J. Dermatol."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1186\/s13023-018-0896-1","article-title":"Meta-analysis of the clinical and immunopathological characteristics and treatment outcomes in epidermolysis bullosa acquisita patients","volume":"13","author":"Iwata","year":"2018","journal-title":"Orphanet J. Rare Dis."},{"key":"ref_25","unstructured":"Baigrie, D., and Nookala, V. (2021, October 03). Bullous Pemphigoid, Available online: https:\/\/www.ncbi.nlm.nih.gov\/books\/NBK535374\/."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"392","DOI":"10.2310\/7750.2014.14004","article-title":"Biopsy Location for Direct Immunofluorescence in Patients with Suspected Bullous Pemphigoid Impacts Probability of a Positive Test Result","volume":"18","author":"Sladden","year":"2014","journal-title":"J. Cutan. Med. Surg."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"831","DOI":"10.1111\/cup.13343","article-title":"The spectrum of histopathologic findings in pemphigoid: Avoiding diagnostic pitfalls","volume":"45","author":"Hodge","year":"2018","journal-title":"J. Cutan. Pathol."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"adv00410","DOI":"10.2340\/00015555-3748","article-title":"Diagnostic Value and Practicability of Serration Pattern Analysis by Direct Immunofluorescence Microscopy in Pemphigoid Diseases","volume":"101","author":"Holtsche","year":"2021","journal-title":"Acta Derm. Venereol."},{"key":"ref_29","unstructured":"Elder, D.E. (2013). Atlas and Synopsis of Levers Histopathology of the Skin, Wolters Kluwer Lippincott Williams & Wilkins."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"269","DOI":"10.1007\/s00403-016-1631-1","article-title":"Evaluation of ELISA testing for BP180 and BP230 as a diagnostic modality for bullous pemphigoid: A clinical experience","volume":"308","author":"Keller","year":"2016","journal-title":"Arch. Dermatol. Res."},{"key":"ref_31","unstructured":"Immunologic Tests: Beutner Labs: United States (2021, July 07). (n.d.). Available online: https:\/\/www.beutnerlabs.com\/."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"268","DOI":"10.4103\/ijd.IJD_196_17","article-title":"Advanced Diagnostic Techniques in Autoimmune Bullous Diseases","volume":"62","author":"Jindal","year":"2017","journal-title":"Indian J. Dermatol."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"748","DOI":"10.1016\/j.jaad.2013.07.009","article-title":"Comparative study of direct and indirect immunofluorescence and of bullous pemphigoid 180 and 230 enzyme-linked immunosorbent assays for diagnosis of bullous pemphigoid","volume":"69","author":"Kostaki","year":"2013","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_34","first-page":"E27","article-title":"Questioning the specificity and sensitivity of ELISA for bullous pemphigoid diagnosis","volume":"99","author":"Muglia","year":"2017","journal-title":"Cutis"},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"1245","DOI":"10.1111\/j.1365-4632.2008.03824.x","article-title":"Enzyme-linked immunosorbent assay (ELISA) and indirect immunofluorescence testing in a bullous pemphigoid and pemphigoid gestationis","volume":"47","author":"Barnadas","year":"2008","journal-title":"Int. J. Dermatol."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"55","DOI":"10.1080\/07853890.2020.1742367","article-title":"Factors associated with the activity and severity of bullous pemphigoid: A review","volume":"52","author":"Liu","year":"2020","journal-title":"Ann. Med."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"24","DOI":"10.2340\/00015555-2473","article-title":"The Reliability, Validity and Responsiveness of Two Disease Scores (BPDAI and ABSIS) for Bullous Pemphigoid: Which One to Use?","volume":"97","author":"Wijayanti","year":"2017","journal-title":"Acta Derm. Venereol."},{"key":"ref_38","unstructured":"Bernett, C.N., Fong, M., and Rosario-Collazo, J.A. (2021). Linear IgA Dermatosis. StatPearls, Available online: https:\/\/www.ncbi.nlm.nih.gov\/books\/NBK526113."},{"key":"ref_39","doi-asserted-by":"crossref","unstructured":"Rasheed, V.S. (2021). Anti-p200 pemphigoid: A review. J. Ski. Sex. Transm. Dis., 1\u20136.","DOI":"10.25259\/JSSTD_35_2021"},{"key":"ref_40","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 List of authors","volume":"346","author":"Joly","year":"2002","journal-title":"N. Engl. J. Med."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"867","DOI":"10.1111\/bjd.13717","article-title":"Management of bullous pemphigoid: The European Dermatology Forum consensus in collaboration with the European Academy of Dermatology and Venereology","volume":"172","author":"Feliciani","year":"2015","journal-title":"Br. J. Dermatol."},{"key":"ref_42","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. Dermatol."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"1630","DOI":"10.1016\/S0140-6736(17)30560-3","article-title":"Doxycycline versus prednisolone as an initial treatment strategy for bullous pemphigoid: A pragmatic, non-inferiority, randomised controlled trial","volume":"389","author":"Williams","year":"2017","journal-title":"Lancet"},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"1536","DOI":"10.1001\/archderm.143.12.1536","article-title":"A Comparison of Oral Methylprednisolone Plus Azathioprine or Mycophenolate Mofetil for the Treatment of Bullous Pemphigoid","volume":"143","author":"Beissert","year":"2007","journal-title":"Arch. Dermatol."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"825","DOI":"10.1067\/mjd.2001.116337","article-title":"Intravenous immunoglobulin therapy for patients with bullous pemphigoid unresponsive to conventional immunosuppressive treatment","volume":"45","author":"Ahmed","year":"2001","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_46","doi-asserted-by":"crossref","unstructured":"Saleh, M., Reedy, M., Torok, H., and Weaver, J. (2021). Successful treatment of bullous pemphigoid with dupilumab: A case and brief review of the literature. Dermatol. Online J., 27.","DOI":"10.5070\/D3274053155"},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"611549","DOI":"10.3389\/fimmu.2020.611549","article-title":"Case Report: Combination of Omalizumab and Dupilumab for Recalcitrant Bullous Pemphigoid","volume":"11","author":"Jafari","year":"2021","journal-title":"Front. Immunol."},{"key":"ref_48","doi-asserted-by":"crossref","unstructured":"Seidman, J.S., Eichenfield, D.Z., and Orme, C.M. (2019). Dupilumab for bullous pemphigoid with intractable pruritus. Dermatol. Online J., 25.","DOI":"10.5070\/D32511046147"},{"key":"ref_49","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-Brazilian Society of Dermatology","volume":"94","author":"Santi","year":"2019","journal-title":"An. Bras. Dermatol."},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"818","DOI":"10.1067\/mjd.2001.113693","article-title":"Emerging treatment for epidermolysis bullosa acquisita","volume":"44","author":"Engineer","year":"2001","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"50","DOI":"10.1080\/09546634.2016.1179711","article-title":"Long-term results of rituximab\u2013intravenous immunoglobulin combination therapy in patients with epidermolysis bullosa acquisita resistant to conventional therapy","volume":"28","author":"Oktem","year":"2017","journal-title":"J. Dermatol. Treat."},{"key":"ref_52","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."}],"container-title":["Medicina"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/1648-9144\/57\/10\/1061\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T07:09:20Z","timestamp":1760166560000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/1648-9144\/57\/10\/1061"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,10,4]]},"references-count":52,"journal-issue":{"issue":"10","published-online":{"date-parts":[[2021,10]]}},"alternative-id":["medicina57101061"],"URL":"https:\/\/doi.org\/10.3390\/medicina57101061","relation":{},"ISSN":["1648-9144"],"issn-type":[{"value":"1648-9144","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,10,4]]}}}