Bullous Pemphigoid: Current and Emerging Therapies
Abstract
Bullous pemphigoid (BP) is the most common autoimmune blistering condition. It preferentially affects the elderly population between the ages of 60 to 80. Younger populations can be afflicted such as in cases involving drug-induced bullous pemphigoid and, rarely, in childhood bullous pemphigoid. The incidence of BP has been rising recently, partly due to increased overall life expectancy.
References
Murrell DF, Daniel BS, Joly P, Borradori L, Amagai M, Hashimoto T, Caux F, Marinovic B, Sinha AA, Hertl M, Bernard P, Sirois D, Cianchini G, Fairley JA, Jonkman MF, Pandya AG, Rubenstein D, Zillikens D, Payne AS, Woodley D, Zambruno G, Aoki V, Pincelli C, Diaz L, Hall RP, Meurer M, Mascaro JM Jr, Schmidt E, Shimizu H, Zone J, Swerlick R, Mimouni D, Culton D, Lipozencic J, Bince B, Grando SA, Bystryn JC, Werth VP. Definitions and outcome measures for bullous pemphigoid: Recommendations by an international panel of experts. J Am Acad Dermatol 2012; 66(3):479-85.
Pratasava V, Sahni VN, Suresh A, Huang S, Are A, Hsu S, Motaparthi K. Bullous pemphigoid and other pemphigoid dermatoses. Medicina (Kaunas) 2021; 57(10):1061.
Patel PM, Jones VA, Murray TN. A review comparing international guidelines for the management of bullous pemphigoid, pemphigoid gestationis, mucous membrane pemphigoid, and epidermolysis bullosa acquisita. Am J Clin Dermatol 2020; 21:557–565.
Bolognia JL, Schaffer JV, Cerroni L. Dermatology. 4th edition, pp 518 Elsevier; 2018.
Feliciani C, Joly P, Jonkman MF, Zambruno G, Zillikens D, Ioannides D, et al. Management of bullous pemphigoid: the European Dermatology Forum consensus in collaboration with the European Academy of Dermatology and Venereology. Br J Dermatol 2015; 172(4):867–77.
Joly P, Roujeau JC, Benichou J, et al. A comparison of oral and topical corticosteroids in patients with bullous pemphigoid. N Engl J Med 2002; 346(5):321–7.
Joly P, Roujeau JC, Benichou J et al. A comparison of two regimens of topical corticosteroids in the treatment of patients with bullous pemphigoid: a multicenter randomized study. J Invest Dermatol 2009; 129:1681–7
Chu J, Bradley M, Marinkovich MP. Topical tacrolimus is a useful adjunctive therapy for bullous pemphigoid. Arch Dermatol 2003; 139:813–15.
Morel P, Guillaume JC. Treatment of bullous pemphigoid with prednisolone only: 0.75 mg/ kg/day versus 1.25 mg/kg/day. A multicenter randomized study. Ann Dermatol Venereol 1984; 111(10):925–8 [in French].
Dreno B, Sassolas B, Lacour P, et al. Methylprednisolone versus prednisolone methylsulfobenzoate in pemphigoid: a comparative multicenter study. Ann Dermatol Venereol 1993; 120(8):518–21.
Venning VA, Taghipour K, Mohd Mustapa MF, Highet AS, Kirtschig G. British Association of Dermatologists’ guidelines for the management of bullous pemphigoid 2012. Br J Dermatol 2012; 1;167(6):1200-14.
Williams, H.C.; Wojnarowska, F.; Kirtschig, G.; Mason, J.; Godec, T.R.; Schmidt, E.; Chalmers, J.R.; Childs, M.; Walton, S.; Harman, K.; et al. Doxycycline versus prednisolone as an initial treatment strategy for bullous pemphigoid: A pragmatic, non-inferiority, randomised controlled trial. Lancet 2017; 389, 1630–1638.
Kalinska-Bienias A, Kowalczyk E, Jagielski P, Kowalewski C, Wozniak K. Tetracycline, nicotinamide, and lesionally administered clobetasol as a therapeutic option to prednisone in patients with bullous pemphigoid: a comparative, retrospective analysis of 106 patients with long-term follow-up. Int J Dermatol 2019; 58(2):172-177.
Salman S, Awad M, Sarsik S, Ibrahim AM, Fathi M, Agha NY, Anis R, El Ashal G, Salem ML. Treatment options for autoimmune bullous dermatoses other than systemic steroids: A systematic review and network meta-analysis. Dermatol Ther 2020; 33(6):e13861.
Burton JL, Harman RR, Peachey RD et al. Azathioprine plus prednisone in treatment of pemphigoid. Br Med J 1978; 2:1190–1.
Guillaume JC, Vaillant L, Bernard P, et al. Controlled trial of azathioprine and plasma exchange in addition to prednisolone in the treatment of bullous pemphigoid. Arch Dermatol 1993; 129(1):49–53.
Heilborn JD, Stahle-Backdahl M, Albertioni F et al. Low-dose oral pulse methotrexate as monotherapy in elderly patients with bullous pemphigoid. J Am Acad Dermatol 1999; 40:741–9.
Bara C, Maillard H, Briand N et al. Methotrexate for bullous pemphigoid: preliminary study. Arch Dermatol 2003; 139:1506–7.
Du-Thanh A, Merlet S, Maillard H et al. Combined treatment with low-dose methotrexate and initial short-term superpotent topical steroids in bullous pemphigoid: an open, multicentre, retrospective study. Br J Dermatol 2011; 165:1337–43.
Kjellman P, Eriksson H, Berg P. A retrospective analysis of patients with bullous pemphigoid treated with methotrexate. Arch Dermatol 2008; 144:612–16.
Grundmann-Kollmann M, Korting HC, Behrens S, et al. Mycophenolate mofetil: a new therapeutic option in the treatment of blistering autoimmune diseases. J Am Acad Dermatol 1999; 40:957–60.
Bohm M, Beissert S, Schwarz T, et al. Bullous pemphigoid treated with mycophenolate mofetil. Lancet 1997; 349:541.
Nousari HC, Griffin WA, Anhalt GJ. Successful therapy for bullous pemphigoid with mycophenolate mofetil. J Am Acad Dermatol 1998; 39:497–8.
Beissert S, Werfel T, Frieling U, et al. A comparison of oral methylprednisolone plus azathioprine or mycophenolate mofetil for the treatment of bullous pemphigoid. Arch Dermatol 2007; 143:1536–42.
Venning VA, Millard PR, Wojnarowska F: Dapsone as first line therapy for bullous pemphigoid. Br J Dermatol 1989; 120:83-92.
Bouscarat F, Chosidow O, Picard-Dahan C et al. Treatment of bullous pemphigoid with dapsone: retrospective study of thirty-six cases. J Am Acad Dermatol 1996; 34:683–4.
Phiamphonsongant T. Dapsone for the treatment of bullous pemphigoid. Asian Pacific J Allergy Immunol 1983; 1:19–21.
Izumi K, Bieber K, Ludwig RJ. Current Clinical Trials in Pemphigus and Pemphigoid. Front Immunol 2019; 10:978.
Seyed Jafari SM, Feldmeyer L, Bossart S, Simon D, Schlapbach C, Borradori L. Case Report: Combination of Omalizumab and Dupilumab for Recalcitrant Bullous Pemphigoid. Front Immunol 2020; 11:611549.
Abdat R, Waldman RA, De Bedout V, et al. Dupilumab as a novel therapy for bullous pemphigoid: a multicenter case series. J Am Acad Dermatol 2020; 83(1):46–52.
Sinha S, Agrawal D, Sardana K, Kulhari A, Malhotra P. Complete Remission in a Patient With Treatment Refractory Bullous Pemphigoid After a Single Dose of Omalizumab. Indian Dermatol Online J 2020; 11:607–11.
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.
Roujeau JC, Guillaume JC, Morel P, et al. Plasma exchange in bullous pemphigoid. Lancet 1984; 2(8401):486–8.
Zhou T, Peng B, Geng S. Emerging Biomarkers and Therapeutic Strategies for Refractory Bullous Pemphigoid. Front Immunol 2021; 4 (12):718073. Am Acad Dermatol. 2020;82(2):398-406.