BACKGROUND: European guidelines propose a 0·5 mg kg-1 per day dose of oral prednisone as initial treatment for bullous pemphigoid (BP). We assessed the safety and efficacy of this regimen depending on BP extent and general condition of the patients. METHODS: In a prospective international study, we consecutively included all patients diagnosed with BP. Patients received a 0·5 mg kg-1 per day dose of prednisone, which was then gradually tapered 15 days after disease control, with the aim of stopping prednisone or maintaining minimal treatment (0·1 mg kg-1 per day) within 6 months after the start of treatment. The two coprimary endpoints were control of disease activity at day 21 and 1-year overall survival. Disease severity was assessed according to the Bullous Pemphigoid Disease Area Index (BPDAI) score. RESULTS: In total, 198 patients were included between 2015 and 2017. The final analysis comprised 190 patients with a mean age of 80·9 (SD 9·1) years. Control of disease activity was achieved at day 21 in 119 patients [62·6%, 95% confidence interval (CI) 55·3-69.5]; 18 of 24 patients (75%, 95% CI 53·3-90·2), 75 of 110 patients (68·8%, 95% CI 59·2-77·3) and 26 of 56 patients (46.4%, 95% CI 33·0-60·3) had mild, moderate and severe BP, respectively (P = 0·0218). A total of 30 patients died during the study. The overall Kaplan-Meier 1-year survival was 82·6% (95% CI 76·3-87·4) corresponding to 90·9%, 83·0% and 80·0% rates in patients with mild, moderate and severe BP, respectively (P = 0·5). Thresholds of 49 points for BPDAI score and 70 points for Karnofsky score yielded maximal Youden index values with respect to disease control at day 21 and 1-year survival, respectively. CONCLUSIONS: A 0·5 mg kg-1 per day dose of prednisone is a valuable therapeutic option in patients with mild or moderate BP whose general condition allows them to be autonomous.

Center for Blistering Diseases Department of Dermatology University Medical Center Groningen Groningen the Netherlands

Department of Biostatistics Rouen University Hospital Rouen France

Department of Dermatology Amiens University Hospital Amiens France

Department of Dermatology Avicenne Hospital University Paris 13 Bobigny France

Department of Dermatology Clermont Ferrand University Hospital Clermont Ferrand France

Department of Dermatology Henri Mondor University Hospital Creteil France

Department of Dermatology Hôpital la Timone Assistance Publique des Hôpitaux de Marseille Marseille France

Department of Dermatology Hospices Civils de Lyon Lyon France

Department of Dermatology Limoges University Hospital Limoges France

Department of Dermatology Medical University of Sofia Sofia Bulgaria

Department of Dermatology Monod General Hospital le Havre France

Department of Dermatology Orleans Hospital Orléans France

Department of Dermatology Rouen University Hospital Center for Autoimmune Bullous Diseases and INSERM U1234 Normandie University Rouen France

Department of Dermatology Saint Louis Hospital Paris France

Department of Dermatology St Anne's Faculty Hospital Brno Czech Republic

Department of Dermatology University of Lübeck Lübeck Germany

Lübeck Institute of Experimental Dermatology University of Lübeck Lübeck Germany

Section of Dermatology Department of Clinical and Experimental Medicine University of Parma Parma Italy

Università degli Studi di Milano Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico Milan Italy

Br J Dermatol. 2021 Dec;185(6):1093-1094. doi: 10.1111/bjd.20753. PubMed

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