Treatment of Bullous Pemphigoid With Dupilumab: A Case Series of 30 Patients
June 2024 | Volume 23 | Issue 6 | e144 | Copyright © June 2024
Published online May 30, 2024
Austinn C. Miller MDa,b, Laurie A. Temiz MDb,c, Susuana Adjei MDb, Miguel A. Duran MDb,d, Jeffrey Sassmannshausen MDe, Arturo Dominguez MDf, Cristina Thomas MDf, Jimmy D. Schmidt MDg, Michael Bernhardt MDa, Coley J. Doolittle-Amieva PA-Ch, Ata S. Moshiri MD MPHh, Anthony J. Thompson BSi, Penelope Pyoung Kim-Lim BSj, Alexzandra V. Mattia BSi, Stephen K. Tyring MD PhD MBAb,k
aDermatology Associates of Tallahassee, Tallahassee, FL
bCenter for Clinical Studies, Webster, TX
cMeharry Medical College, Nashville, TN
dUniversidad Autonoma de Campeche, Campeche, Mexico
eThree Rivers Dermatology, Fort Wayne, IN
fDepartment of Dermatology, University of Texas Southwestern Medical Center, Dallas, TX
gDermSurgery Associate, Houston, TX
hDivision of Dermatology, University of Washington, Seattle, WA
iFlorida State University College of Medicine, Tallahassee, FL
jUniversity of California, Davis School of Medicine, Davis, CA
kDepartment of Dermatology, University of Texas Health Science Center at Houston, Houston, TX
Abstract
Bullous pemphigoid is often difficult to treat with the limited therapies available. Here, we describe clinical outcomes among 30 adults with bullous pemphigoid patients treated with dupilumab. We performed a multicenter, retrospective case series between March 2020 to August 2022. Patients received a loading dose of dupilumab 600 mg, followed by 300 mg maintenance dose with varying administration frequency tailored to individual patient response. All patients experienced at least some improvement in blister formation and pruritus, with 23 (76.7%) of patients demonstrating either complete clearance of blistering or marked response. Complete clearance of pruritus or marked response was noted in 25 (83.3%) of patients. Eight patients were effectively maintained solely on dupilumab. One (3.3%) patient reported an injection site reaction. Thirty patients represent a small sample, however, to our knowledge, this is the second largest group of BP treated with dupilumab. Furthermore, we provide an understandable framework for clinicians outside of academics to follow and assess treatment responses in their BP patients treated with dupilumab. Dupilumab should be considered as a therapeutic option in patients with bullous pemphigoid given its ability to induce sustained blistering and pruritus response in both typical and refractory cases while maintaining a favorable safety profile.
J Drugs Dermatol. 2024;23(6):e144-e148. doi:10.36849/JDD.8258e
INTRODUCTION
Bullous pemphigoid (BP) is the most common autoimmune subepidermal blistering disease, propagated by autoantibodies against BP antigen 230 (BPAG1) and BP antigen 180 (BPAG2).1 Classically, it presents with intensely pruritic eczematous or urticarial plaques and tense bullae in elderly patients.1 Less commonly, non-bullous variants occur with eczematous or urticarial plaques alone.1 Diagnosis relies upon histopathology, direct and indirect immunofluorescence microscopy (DIF/IIF), as well as anti-BP180/BP230 enzyme-linked immunosorbent assays (ELISAs).1
Mild or localized disease may be controlled with topical corticosteroids. However, more severe disease is often difficult to treat. Systemic immunosuppression with oral corticosteroids remains the first-line treatment for widespread cases, often leading to detrimental side effects, although effective. Second- and third-line agents for recalcitrant disease include a plethora of immunosuppressants such as mycophenolate mofetil, methotrexate, azathioprine, cyclophosphamide, and tetracyclines. Recalcitrant cases are often treated with IVIG and rituximab.
