Rana Abdat1, Reid A Waldman2, Valeria de Bedout3, Annette Czernik4, Michael Mcleod1, Brett King5, Samantha Gordon1, Razzaque Ahmed1, Anna Nichols3, Marti Rothe2, David Rosmarin6. 1. Department of Dermatology, Tufts Medical Center, Boston, Massachusetts. 2. Department of Dermatology, University of Connecticut Health Center, Farmington, Connecticut. 3. Dr. Phillip Frost Department of Dermatology & Cutaneous Surgery, University of Miami Health System, Miami, Florida. 4. Department of Dermatology, Mount Sinai Health System, New York, New York. 5. Department of Dermatology, Yale School of Medicine, New Haven, Connecticut. 6. Department of Dermatology, Tufts Medical Center, Boston, Massachusetts. Electronic address: rosmarin@gmail.com.
Abstract
BACKGROUND: Bullous pemphigoid (BP) is an autoimmune blistering disorder occurring mostly in the elderly that lacks adequate treatments. OBJECTIVE: To describe our experience using dupilumab in a series of patients with BP. METHODS: This is a case series of patients from 5 academic centers receiving dupilumab for BP. Patients were eligible if they had a clinical diagnosis of BP confirmed by lesional skin biopsy evaluated by one of more of the following: hematoxylin and eosin staining, direct immunofluorescence, or enzyme-linked immunosorbent assay for BP180 or BP230, or both. RESULTS: We identified 13 patients. Patients were an average age of 76.8 years, and the average duration of BP before dupilumab initiation was 28.8 months (range, 1-60 months). Disease clearance or satisfactory response was achieved in 92.3% (12 of 13) of the patients. Satisfactory response was defined as clinician documentation of disease improvement and patient desire to stay on the medication without documentation of disease clearance. Total clearance of the BP was achieved in 53.8% (7of 13) of patients No adverse events were reported. LIMITATIONS: Include small sample size, lack of a control group, lack of a standardized assessment tool, and lack of standardized safety monitoring. CONCLUSION: Dupilumab may be an additional treatment for BP, leading to disease clearance or satisfactory response in 92.3% of patients, including in those in whom previous conventional therapy had failed.
BACKGROUND: Bullous pemphigoid (BP) is an autoimmune blistering disorder occurring mostly in the elderly that lacks adequate treatments. OBJECTIVE: To describe our experience using dupilumab in a series of patients with BP. METHODS: This is a case series of patients from 5 academic centers receiving dupilumab for BP. Patients were eligible if they had a clinical diagnosis of BP confirmed by lesional skin biopsy evaluated by one of more of the following: hematoxylin and eosin staining, direct immunofluorescence, or enzyme-linked immunosorbent assay for BP180 or BP230, or both. RESULTS: We identified 13 patients. Patients were an average age of 76.8 years, and the average duration of BP before dupilumab initiation was 28.8 months (range, 1-60 months). Disease clearance or satisfactory response was achieved in 92.3% (12 of 13) of the patients. Satisfactory response was defined as clinician documentation of disease improvement and patient desire to stay on the medication without documentation of disease clearance. Total clearance of the BP was achieved in 53.8% (7of 13) of patients No adverse events were reported. LIMITATIONS: Include small sample size, lack of a control group, lack of a standardized assessment tool, and lack of standardized safety monitoring. CONCLUSION:Dupilumab may be an additional treatment for BP, leading to disease clearance or satisfactory response in 92.3% of patients, including in those in whom previous conventional therapy had failed.
Authors: Hideyuki Ujiie; David Rosmarin; Michael P Schön; Sonja Ständer; Katharina Boch; Martin Metz; Marcus Maurer; Diamant Thaci; Enno Schmidt; Connor Cole; Kyle T Amber; Dario Didona; Michael Hertl; Andreas Recke; Hanna Graßhoff; Alexander Hackel; Anja Schumann; Gabriela Riemekasten; Katja Bieber; Gant Sprow; Joshua Dan; Detlef Zillikens; Tanya Sezin; Angela M Christiano; Kerstin Wolk; Robert Sabat; Khalaf Kridin; Victoria P Werth; Ralf J Ludwig Journal: Front Med (Lausanne) Date: 2022-06-09
Authors: Yale Liu; Hao Wang; Mark Taylor; Christopher Cook; Alejandra Martínez-Berdeja; Jeffrey P North; Paymann Harirchian; Ashley A Hailer; Zijun Zhao; Ruby Ghadially; Roberto R Ricardo-Gonzalez; Roy C Grekin; Theodora M Mauro; Esther Kim; Jaehyuk Choi; Elizabeth Purdom; Raymond J Cho; Jeffrey B Cheng Journal: Sci Immunol Date: 2022-04-15