{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,28]],"date-time":"2026-07-28T19:09:01Z","timestamp":1785265741401,"version":"3.55.0"},"reference-count":23,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2020,11,20]],"date-time":"2020-11-20T00:00:00Z","timestamp":1605830400000},"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. Med."],"abstract":"<jats:p><jats:bold>Background:<\/jats:bold> Treatment regimens for pemphigoid gestationis (PG) are non-standardized, with most evidence derived from individual case reports or small series.<\/jats:p><jats:p><jats:bold>Objectives:<\/jats:bold> To systematically review current literature on treatments and clinical outcomes of PG and to establish recommendations on its therapeutic management.<\/jats:p><jats:p><jats:bold>Methods:<\/jats:bold> An a priori protocol was designed based on PRISMA guidelines. PubMed, Scopus, and Web of Science databases were searched for English-language articles detailing PG treatments and clinical outcomes, published between 1970 and March 2020.<\/jats:p><jats:p><jats:bold>Results:<\/jats:bold> In total, 109 articles including 140 PG patients were analyzed. No randomized controlled trials or robust observational studies detailing PG treatment were found. Systemic corticosteroids \u00b1 topical corticosteroids and\/or antihistamines were the most frequently prescribed treatment modality (<jats:italic>n<\/jats:italic> = 74\/137; 54%). Complete remission was achieved by 114\/136 (83.8%) patients. Sixty-four patients (45.7%) were given more than one treatment modality due to side effects or ineffectiveness. Leaving aside topical corticosteroids as monotherapy \u00b1 antihistamines in patients with mild disease, systemic corticosteroids \u00b1 topical corticosteroids and\/or antihistamines led to complete remission in the highest proportion of patients (83%), while steroid-sparing treatments \u00b1 topical corticosteroids and\/or antihistamines were associated with the lowest proportion of flares (55.5%).<\/jats:p><jats:p><jats:bold>Limitations:<\/jats:bold> The review has been drafted based on a limited number of single case reports and small case series. Underreporting\/underdiagnosis of patients with mild-to-moderate PG, partial\/absent follow-up, absence of precise description of neonatal outcomes and lack of validated objective scores for measuring disease severity are other limitations of our study. Our systematic review was affected by publication bias.<\/jats:p><jats:p><jats:bold>Conclusion:<\/jats:bold> Systemic corticosteroids are the most frequently used treatment for PG. Whilst most patients achieve complete remission, many of them have refractory\/persistent disease requiring multiple lines of therapy. Therefore, we provided an algorithm for PG treatment integrating the results of this systematic review with current knowledge available for bullous pemphigoid. High-quality studies will further help assess the effectiveness of different treatment options for PG.<\/jats:p>","DOI":"10.3389\/fmed.2020.604945","type":"journal-article","created":{"date-parts":[[2020,11,20]],"date-time":"2020-11-20T18:29:47Z","timestamp":1605896987000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":35,"title":["A Systematic Review of Treatment Options and Clinical Outcomes in Pemphigoid Gestationis"],"prefix":"10.3389","volume":"7","author":[{"given":"Giovanni","family":"Genovese","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Federica","family":"Derlino","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amilcare","family":"Cerri","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chiara","family":"Moltrasio","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Simona","family":"Muratori","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emilio","family":"Berti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Angelo Valerio","family":"Marzano","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2020,11,20]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"395","DOI":"10.1016\/j.jaad.2005.12.012","article-title":"The specific dermatoses of pregnancy revisited and reclassified: results of a retrospective two-center study on 505 pregnant patients","volume":"54","author":"Ambros-Rudolph","year":"2006","journal-title":"J Am Acad Dermatol."},{"key":"B2","doi-asserted-by":"publisher","first-page":"256","DOI":"10.23736\/S0392-0488.18.06153-9","article-title":"Autoimmune bullous diseases during pregnancy: insight into pathogenetic mechanisms and clinical features","volume":"154","author":"Feliciani","year":"2019","journal-title":"G Ital Dermatol Venereol."},{"key":"B3","doi-asserted-by":"publisher","first-page":"441","DOI":"10.2147\/CCID.S128144","article-title":"Pemphigoid gestationis: current perspectives","volume":"10","author":"S\u00e4vervall","year":"2017","journal-title":"Clin Cosmet Investig Dermatol."},{"key":"B4","doi-asserted-by":"publisher","first-page":"138","DOI":"10.1016\/j.ejogrb.2009.05.012","article-title":"Pemphigoid gestationis: current insights into pathogenesis and treatment","volume":"145","author":"Semkova","year":"2009","journal-title":"Eur J Obstet Gynecol Reprod Biol."},{"key":"B5","doi-asserted-by":"publisher","first-page":"391","DOI":"10.1007\/s40257-018-0342-0","article-title":"Treatment of autoimmune bullous disorders in pregnancy","volume":"19","author":"Kushner","year":"2018","journal-title":"Am J Clin Dermatol."},{"key":"B6","doi-asserted-by":"publisher","first-page":"264","DOI":"10.1016\/j.annder.2011.01.015","article-title":"Soci\u00e9t\u00e9 Fran\u00e7aise de Dermatologie. Pemphigoid gestationis. Guidelines for the diagnosis and treatment. Centres de r\u00e9f\u00e9rence des maladies bulleuses auto-immunes. Soci\u00e9t\u00e9 Fran\u00e7aise de Dermatologie","volume":"138","author":"Ingen-Housz-Oro","year":"2011","journal-title":"Ann Dermatol Venereol."},{"key":"B7","doi-asserted-by":"publisher","first-page":"b2700","DOI":"10.1371\/journal.pmed.1000100","article-title":"The PRISMA statement for reporting systematic reviews and meta-analyses of studies that evaluate healthcare interventions: explanation and elaboration","volume":"339","author":"Liberati","year":"2009","journal-title":"BMJ."},{"key":"B8","doi-asserted-by":"publisher","first-page":"479","DOI":"10.1016\/j.jaad.2011.06.032","article-title":"Definitions and outcome measures for bullous pemphigoid: recommendations by an international panel of experts","volume":"66","author":"Murrell","year":"2012","journal-title":"J Am Acad Dermatol."},{"key":"B9","doi-asserted-by":"publisher","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":"B10","doi-asserted-by":"publisher","first-page":"113","DOI":"10.1016\/j.annder.2011.01.009","article-title":"Chronic pemphigoid gestationis: comparative clinical and immunopathological study of 10 patients","volume":"206","author":"Boulinguez","year":"2003","journal-title":"Dermatology."},{"key":"B11","doi-asserted-by":"publisher","first-page":"1222","DOI":"10.1111\/j.1365-2133.2009.09086.x","article-title":"Pemphigoid gestationis: early onset and blister formation are associated with adverse pregnancy outcomes","volume":"160","author":"Chi","year":"2009","journal-title":"Br J Dermatol."},{"key":"B12","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1016\/j.intimp.2015.02.038","article-title":"Positive clinical outcome with IVIg as monotherapy in recurrent pemphigoid gestationis","volume":"26","author":"Nguyen","year":"2015","journal-title":"Int Immunopharmacol."},{"key":"B13","doi-asserted-by":"publisher","first-page":"1073","DOI":"10.1111\/j.1468-3083.2010.03925.x","article-title":"Use of intravenous immunoglobulin therapy during pregnancy in patients with pemphigus vulgaris","volume":"25","author":"Ahmed","year":"2011","journal-title":"J Eur Acad Dermatol Venereol."},{"key":"B14","doi-asserted-by":"publisher","first-page":"593","DOI":"10.2340\/00015555-2032","article-title":"Cyclosporine treatment in severe gestational pemphigoid","volume":"95","author":"Huilaja","year":"2015","journal-title":"Acta Derm Venereol."},{"key":"B15","doi-asserted-by":"publisher","first-page":"20","DOI":"10.1556\/1646.8.2016.1.3","article-title":"A resistant case of pemphigus gestationis successfully treated with cyclosporine","volume":"8","author":"\u00d6zdemir","year":"2016","journal-title":"Interv Med Appl Sci."},{"key":"B16","doi-asserted-by":"publisher","first-page":"e206","DOI":"10.1111\/jdv.13962","article-title":"Pemphigoid gestationis: a successful preventive treatment by rituximab","volume":"31","author":"Tourte","year":"2017","journal-title":"J Eur Acad Dermatol Venereol."},{"key":"B17","doi-asserted-by":"publisher","first-page":"388","DOI":"10.1111\/j.1365-2133.2007.07982.x","article-title":"Severe persistent pemphigoid gestationis: long-term remission with rituximab","volume":"157","author":"Cianchini","year":"2007","journal-title":"Br J Dermatol."},{"key":"B18","doi-asserted-by":"publisher","first-page":"e331","DOI":"10.1111\/jdv.14119","article-title":"Pregnancy outcome after rituximab treatment before conception in patients affected by severe pemphigus vulgaris\/superficialis","volume":"31","author":"Vassallo","year":"2017","journal-title":"J Eur Acad Dermatol Venereol."},{"key":"B19","doi-asserted-by":"publisher","first-page":"751","DOI":"10.1080\/09546634.2016.1255302","article-title":"Rituximab treatment of pemphigus in women of childbearing age: experience with two patients","volume":"28","author":"Lake","year":"2017","journal-title":"J Dermatolog Treat."},{"key":"B20","first-page":"1401","article-title":"Safety of azathioprine use during pregnancy","volume":"57","author":"Natekar","year":"2011","journal-title":"Can Fam Physician."},{"key":"B21","doi-asserted-by":"publisher","first-page":"443","DOI":"10.1016\/0016-5085(90)91027-4","article-title":"Safety of azathioprine in pregnancy in inflammatory bowel disease","volume":"99","author":"Alstead","year":"1990","journal-title":"Gastroenterology."},{"key":"B22","doi-asserted-by":"publisher","first-page":"633","DOI":"10.2165\/00002018-200427090-00002","article-title":"Dapsone therapy for malaria during pregnancy: maternal and fetal outcomes","volume":"27","author":"Brabin","year":"2004","journal-title":"Drug Saf."},{"key":"B23","doi-asserted-by":"publisher","first-page":"12414","DOI":"10.1111\/dth.12414","article-title":"Leprosy treatment during pregnancy and breastfeeding: A case report and brief review of literature","volume":"30","author":"Ozturk","year":"2017","journal-title":"Dermatol Ther."}],"container-title":["Frontiers in Medicine"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fmed.2020.604945\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2020,11,20]],"date-time":"2020-11-20T18:29:49Z","timestamp":1605896989000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fmed.2020.604945\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,11,20]]},"references-count":23,"alternative-id":["10.3389\/fmed.2020.604945"],"URL":"https:\/\/doi.org\/10.3389\/fmed.2020.604945","relation":{},"ISSN":["2296-858X"],"issn-type":[{"value":"2296-858X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,11,20]]},"article-number":"604945"}}