Reviews
Early Access
The efficacy and safety of intravenous immunoglobulin in pemphigus vulgaris and bullous pemphigoid: a systematic review and meta-analysis
Publisher's note
All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.
All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.
Published: 7 September 2026
10
Views
0
Downloads
Authors
Autoimmune blistering diseases (AIBDs) encompass various skin conditions that involve the production of autoantibodies that attach to specific antigens found in the skin and mucous membranes. This systematic review examined the efficacy and safety of intravenous immunoglobulin (IVIG) therapy for AIBDs. The search included PubMed, Science Direct, Google Scholar, Wiley, and EBSCO using the following keyword combinations: (Pemphigus Vulgaris OR Bullous Pemphigoid OR Autoimmune Blistering Disease) AND (Intravenous Immunoglobulin OR IVIG) AND (Efficacy OR Long-term Outcomes) AND (Safety OR Adverse Effects OR Quality of Life). Of 617 papers, 12 articles were considered suitable for the systematic review and extracted through the database search. A meta-analysis demonstrated an efficacy of 85.0% (95% confidence interval [CI]: 75.5-92.3, p<0.001) for IVIG therapy in AIBDs. Subgroup analyses showed relapse rates of 18.2% (95% CI: 9.3-27.0, p<0.001) and adverse event rates of 48.0% (95% CI: 28.7-67.3, p<0.001). Rapid clinical responses occurred within 1-4 weeks, with sustained remission lasting up to 44 months. Corticosteroid use was reduced by 76-90%, and autoantibody titers declined significantly. While adverse events were common (26-88% of patients), most were mild, with severe complications (<5%). IVIG is effective for AIBDs, offering rapid control, durable remission, and significant steroid reduction. Standardized protocols for dosing and monitoring are needed, and future research should focus on large-scale trials to confirm long-term benefits.
Downloads
Download data is not yet available.
Citations
1. Kasperkiewicz M, Ellebrecht CT, Takahashi H, et al. Pemphigus. Nat Rev Dis Primers 2017;3:17026.
2. Schmidt E, Zillikens D. Pemphigoid diseases. Lancet Lond Engl 2013;381:320-32.
3. Schmidt E, Zillikens D. The diagnosis and treatment of autoimmune blistering skin diseases. Dtsch Ärzteblatt Int 2011;108:399-405.
4. Schmidt E, Zillikens D. Modern diagnosis of autoimmune blistering skin diseases. Autoimmun Rev 2010;10:84-9.
5. Bertram F, Bröcker E-B, Zillikens D, Schmidt E. Prospective analysis of the incidence of autoimmune bullous disorders in Lower Franconia, Germany. J Dtsch Dermatol Ges J Ger Soc Dermatol 2009;7:434-40.
6. Försti A-K, Jokelainen J, Timonen M, Tasanen K. Increasing incidence of bullous pemphigoid in Northern Finland: a retrospective database study in Oulu University Hospital. Br J Dermatol 2014;171:1223-6.
7. Langan SM, Smeeth L, Hubbard R, et al. Bullous pemphigoid and pemphigus vulgaris–incidence and mortality in the UK: population-based cohort study. BMJ 2008;337:a180.
8. Meyer N, Misery L. Geoepidemiologic considerations of auto-immune pemphigus. Autoimmun Rev 2010;9:A379-82.
9. Kridin K. Pemphigus group: overview, epidemiology, mortality, and comorbidities. Immunol Res 2018;66:255-70.
10. Kridin K, Hübner F, Recke A, et al. The burden of neurological comorbidities in six autoimmune bullous diseases: a population-based study. J Eur Acad Dermatol Venereol 2021;35:2074-8.
11. Kianfar N, Dasdar S, Mahmoudi H, Daneshpazhooh M. Burden of pemphigus vulgaris with a particular focus on women: a review. Int J Womens Dermatol 2022;8:e056.
12. Yasir M, Goyal A, Bansal P, Sonthalia S. Corticosteroid Adverse Effects. StatPearls Publishing; 2018.
13. Mazaherpour E, Kianfar N, Dasdar S, et al. Applicability of glucocorticoid toxicity index in pemphigus: comparison between two groups of rituximab-treated and rituximab-naïve patients. Dermatol Ther 2022;35:e15902.
14. Bilgic A, Murrell DF. What is novel in the clinical management of pemphigus. Expert Rev Clin Pharmacol 2019;12:973-80.
15. Norris PAA, Kaur G, Lazarus AH. New insights into IVIg mechanisms and alternatives in autoimmune and inflammatory diseases. Curr Opin Hematol 2020;27:392-8.
16. Hoffmann JHO, Enk AH. High-dose intravenous immunoglobulin in skin autoimmune disease. Front Immunol 2019;10:1090.
17. Shock A, Humphreys D, Nimmerjahn F. Dissecting the mechanism of action of intravenous immunoglobulin in human autoimmune disease: lessons from therapeutic modalities targeting Fcγ receptors. J Allergy Clin Immunol 2020;146:492-500.
18. Page MJ, Moher D. Evaluations of the uptake and impact of the Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) Statement and extensions: a scoping review. Syst Rev 2017;6:263.
19. Sterne JAC, Savović J, Page MJ, et al. RoB 2: a revised tool for assessing risk of bias in randomised trials. BMJ 2019;366:l4898.
20. Slim K, Nini E, Forestier D, et al. Methodological index for non-randomized studies (MINORS): development and validation of a new instrument. Br J Surg 2003;90:227-32.
21. Wallace BC, Dahabreh IJ, Trikalinos TA, et al. Closing the Gap between Methodologists and End-Users: R as a Computational Back-End. J Stat Soft 2012;49:1-15.
22. Ahmed AR. Intravenous immunoglobulin therapy for patients with bullous pemphigoid unresponsive to conventional immunosuppressive treatment. J Am Acad Dermatol 2001;45:825-35.
23. Baum S, Scope A, Barzilai A, et al. The role of IVIg treatment in severe pemphigus vulgaris. J Eur Acad Dermatol Venereol 2006;20:548-52.
24. Segura S, Iranzo P, Martínez-de Pablo I, et al. High-dose intravenous immunoglobulins for the treatment of autoimmune mucocutaneous blistering diseases: evaluation of its use in 19 cases. J Am Acad Dermatol 2007;56:960-7.
25. Xiao T, Li B, Wang YK, et al. Childhood bullous pemphigoid treated by i.v. immunoglobulin. J Dermatol 2007;34:650-3.
26. Amagai M, Ikeda S, Shimizu H, et al; Pemphigus Study Group. A randomized double-blind trial of intravenous immunoglobulin for pemphigus. J Am Acad Dermatol 2009;60:595-603.
27. Arnold DF, Burton J, Shine B, et al. An ‘n‐of‐1’placebo‐controlled crossover trial of intravenous immunoglobulin as adjuvant therapy in refractory pemphigus vulgaris. Br J Dermatol 2009;160:1098-102.
28. Asarch A, Razzaque Ahmed A. Treatment of juvenile pemphigus vulgaris with intravenous immunoglobulin therapy. Pediatr Dermatol 2009;26:197-202.
29. Gaitanis G, Alexis I, Pelidou SH, et al. High-dose intravenous immunoglobulin in the treatment of adult patients with bullous pemphigoid. Eur J Dermatol 2012;22:363-9.
30. Seidling V, Hoffmann JH, Enk AH, Hadaschik EN. Analysis of high-dose intravenous immunoglobulin therapy in 16 patients with refractory autoimmune blistering skin disease: high efficacy and no serious adverse events. Acta Derm Venereol 2013;93:346-9.
31. Amagai M, Ikeda S, Hashimoto T, et al; Bullous Pemphigoid Study Group. A randomized double-blind trial of intravenous immunoglobulin for bullous pemphigoid. J Dermatol Sci 2017;85:77-84.
32. Yu Y, Furuta J, Watanabe R, et al. Case of bullous pemphigoid who achieved a long-term remission by a single course of high-dose i.v. immunoglobulin monotherapy. J Dermatol 2020;47:e225-7.
33. Spałek MM, Bowszyc-Dmochowska M, Dmochowski M. Intravenous immunoglobulin for autoimmune bullous diseases: a case series from a Central European referral center. Medicina 2023;59:1265.
34. Kianfar N, Dasdar S, Daneshpazhooh M, et al. A systematic review on efficacy, safety and treatment durability of intravenous immunoglobulin in autoimmune bullous dermatoses: Special focus on indication and combination therapy. Exp Dermatol 2023;32:934-44.
How to Cite
1.
Alwusaidi H, Alahmadi M, Alghamdi S, Almulla S, Khoj Z, Al Mashhrawi Y, et al. The efficacy and safety of intravenous immunoglobulin in pemphigus vulgaris and bullous pemphigoid: a systematic review and meta-analysis. Dermatol Reports [Internet]. 2026 Sep. 7 [cited 2026 Sep. 9];. Available from: https://journals.pagepress.net/dr/article/view/10560
Copyright (c) 2026 the Author(s)
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
