BACKGROUND: Bullous pemphigoid (BP) is the most common autoimmune subepidermal blistering disease of the skin and mucous membranes. This disease typically affects the elderly and presents with itch and localized or, most frequently, generalized bullous lesions. A subset of patients only develops excoriations, prurigo-like lesions, and eczematous and/or urticarial erythematous lesions. The disease, which is significantly associated with neurological disorders, has high morbidity and severely impacts the quality of life. OBJECTIVES AND METHODOLOGY: The Autoimmune blistering diseases Task Force of the European Academy of Dermatology and Venereology sought to update the guidelines for the management of BP based on new clinical information, and new evidence on diagnostic tools and interventions. The recommendations are either evidence-based or rely on expert opinion. The degree of consent among all task force members was included. RESULTS: Treatment depends on the severity of BP and patients' comorbidities. High-potency topical corticosteroids are recommended as the mainstay of treatment whenever possible. Oral prednisone at a dose of 0.5 mg/kg/day is a recommended alternative. In case of contraindications or resistance to corticosteroids, immunosuppressive therapies, such as methotrexate, azathioprine, mycophenolate mofetil or mycophenolate acid, may be recommended. The use of doxycycline and dapsone is controversial. They may be recommended, in particular, in patients with contraindications to oral corticosteroids. B-cell-depleting therapy and intravenous immunoglobulins may be considered in treatment-resistant cases. Omalizumab and dupilumab have recently shown promising results. The final version of the guideline was consented to by several patient organizations. CONCLUSIONS: The guidelines for the management of BP were updated. They summarize evidence- and expert-based recommendations useful in clinical practice.

1st Department of Dermatology Venereology Hospital of Skin and Venereal Diseases Aristotle University Medical School Thessaloniki Greece

2nd Department of Dermatology Autoimmune Bullous Diseases Unit Aristotle University School of Medicine Papageorgiou General Hospital Thessaloniki Greece

Abteilung Dermatologie Venerologie und Allergologie Lehrkrankenhaus der Medizinischen Universität Wien Austria

Autoimmune Blistering Dermatoses Section Department of Dermatology Poznan University of Medical Sciences Poznan Poland

Center for Research on Inflammation of the Skin University of Lübeck Lübeck Germany

Department Dermatology and Immunodermatology Medical University of Warsaw Warsaw Poland

Department of Dermatology Aarhus University Hospital Aarhus Denmark

Department of Dermatology Allergy and Dermatosurgery Helios University Hospital Wuppertal University Witten Herdecke Germany

Department of Dermatology and Allergology Ludwig Maximilian University Munich Germany

Department of Dermatology and Allergology Philipps University Marburg Germany

Department of Dermatology and Referral Center for Autoimmune Bullous Diseases Groupe Hospitalier Paris Seine Saint Denis AP HP and University Paris 13 Bobigny France

Department of Dermatology and Venereology Akdeniz University Faculty of Medicine Antalya Turkey

Department of Dermatology and Venereology School of Medicine University Hospital Centre Zagreb University of Zagreb Zagreb Croatia

Department of Dermatology Autoimmune Bullous Diseases Research Center Razi Hospital Tehran University of Medical Sciences Tehran Iran

Department of Dermatology Carl Gustav Carus University Hospital Technische Universität Dresden Dresden Germany

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

Department of Dermatology Hospital Clínic de Barcelona Universitat de Barcelona Barcelona Spain

Department of Dermatology Inselspital Bern University Hospital Bern Switzerland

Department of Dermatology Medical University of Sofia Sofia Bulgaria

Department of Dermatology Medical University Sofia Bulgaria

Department of Dermatology Ospedale Classificato Cristo Re Rome Italy

Department of Dermatology Rambam Health Care Campus Haifa Israel

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

Department of Dermatology School of Medicine Koç University Istanbul Turkey

Department of Dermatology St George Hospital University of New South Wales Sydney New South Wales Australia

Department of Dermatology the PEDEGO Research Unit University of Oulu and Medical Research Center Oulu Oulu University Hospital Oulu Finland

Department of Dermatology University of Lübeck Lübeck Germany

Department of Dermatology University of Münster Münster Germany

Department of Dermatology Venereology and Allergology Innsbruck Medical University Innsbruck Austria

Department of Dermatology Venereology and Allergology University Hospital Würzburg Würzburg Germany

Department of Dermatology Venereology and Allergy Clinical Center Landstrasse Academic Teaching Hospital of the Medical University of Vienna Vienna Austria

Department of Dermatology Venereology and Dermatooncology Semmelweis University Budapest Hungary

Department of Dermatology Venereology and Leprology Postgraduate Institute of Medical Education and Research Chandigarh India

Department of Dermatology Xijing Hospital 4th Military Medical University Xi'an China

Department of Dermatovenereology Masaryk University University Hospital St Anna Brno

Department of Dermatovenereology University Hospital Brno Brno Czech Republic

Department of Health Sciences Section of Dermatology AUSL Toscana Centro Rare Diseases Unit European Reference Network Skin Member University of Florence Italy

Department of Medicine Yong Loo Lin School of Medicine National University of Singapore Singapore

Department of Oral Medicine St John's Institute of Dermatology Guy's and St Thomas' NHS Foundation Trust London UK

Department of Pathophysiology and Transplantation Università degli Studi di Milano Milan Italy

Dermatology Unit Department of Medicine and Surgery University Hospital University of Parma Italy

Dermatology Unit Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico Milan Italy

DermoLab Institute of Dermatology University of Modena and Reggio Emilia Modena Italy

Division of Dermatology Tel Aviv Sourasky Medical Center and Department of Human Molecular Genetics and Biochemistry Sackler Faculty of Medicine Tel Aviv University Tel Aviv Israel

Division of Genetics and Molecular Medicine King's College London Guy's Hospital London UK

Genetics and Rare Diseases Research Division Bambino Gesù Children's Hospital IRCCS Rome Italy

Laboratory of Molecular and Cell Biology Istituto Dermopatico dell'Immacolata IDI IRCCS Rome Italy

Lübeck Institute of Experimental Dermatology University of Lübeck Lübeck Germany

Medical Faculty The Sigmund Freud Private University Vienna Austria

National Skin Centre Singapore Singapore

Rappaport Faculty of Medicine Technion Israel Institute of Technology Haifa Israel

Section of Dermatology Department of Health Sciences University of Florence Florence Italy

St John's Institute of Dermatology Viapath Analytics LLP St Thomas' Hospital London UK

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