BACKGROUND: Several European countries recently developed international diagnostic and management guidelines for pemphigus, which have been instrumental in the standardization of pemphigus management. OBJECTIVE: We now present results from a subsequent Delphi consensus to broaden the generalizability of the recommendations. METHODS: A preliminary survey, based on the European Dermatology Forum and the European Academy of Dermatology and Venereology guidelines, was sent to a panel of international experts to determine the level of consensus. The results were discussed at the International Bullous Diseases Consensus Group in March 2016 during the annual American Academy of Dermatology conference. Following the meeting, a second survey was sent to more experts to achieve greater international consensus. RESULTS: The 39 experts participated in the first round of the Delphi survey, and 54 experts from 21 countries completed the second round. The number of statements in the survey was reduced from 175 topics in Delphi I to 24 topics in Delphi II on the basis of Delphi results and meeting discussion. LIMITATIONS: Each recommendation represents the majority opinion and therefore may not reflect all possible treatment options available. CONCLUSIONS: We present here the recommendations resulting from this Delphi process. This international consensus includes intravenous CD20 inhibitors as a first-line therapy option for moderate-to-severe pemphigus.

1st Department of Dermatology Aristotle University Thessaloniki Greece

2nd Dermatology Department Aristotle University School of Medicine Papageorgiou General Hospital Thessaloniki Greece

Clinica Dermatologica Universita' Di Parma Parma Italy

Corporal Michael J Crescenz Veterans Affairs Medical Center Philadelphia Pennsylvania; Department of Dermatology at the Perelman School of Medicine University of Pennsylvania Philadelphia Pennsylvania

Departamento de Dermatologia Universidade de Sao Paulo Sao Paulo Brazil

Department of Dermatology and Allergology University Hospital Philipps Universität Marburg Marburg Germany

Department of Dermatology and Immunodermatology Medical University of Warsaw Warsaw Poland

Department of Dermatology and the Murdough Family Center for Psoriasis University Hospitals Case Medical Center Cleveland Ohio

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

Department of Dermatology and Venereology Medical Faculty University of Medicine Sofia Bulgaria

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

Department of Dermatology at the Perelman School of Medicine University of Pennsylvania Philadelphia Pennsylvania

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

Department of Dermatology Avicenne Hospital University Paris 13 Bobigny France

Department of Dermatology Department of Histology Reference Center for Autoimmune Bullous Diseases Avicenne Hospital University Paris 13 Bobigny France

Department of Dermatology Emory University School of Medicine Atlanta Georgia

Department of Dermatology Faculty of Medicine Cairo University Cairo Egypt

Department of Dermatology Gangnam Severance Hospital Yonsei University College of Medicine Seoul Korea

Department of Dermatology Hokkaido University Graduate School of Medicine Sapporo Japan

Department of Dermatology Hospital Clínic Universitat de Barcelona Barcelona Spain

Department of Dermatology Icahn School of Medicine at Mount Sinai New York New York

Department of Dermatology Jacobs School of Medicine and Biomedical Sciences University at Buffalo Buffalo New York

Department of Dermatology Keio University School of Medicine Tokyo Japan

Department of Dermatology Mayo Clinic College of Medicine Rochester Minnesota

Department of Dermatology Rabin Medical Center Beilinson Campus Petach Tikva Israel; Sackler School of Medicine Tel Aviv University Tel Aviv Israel

Department of Dermatology Reims University Hospital University of Champagne Ardenne Reims France

Department of Dermatology Rouen University Hospital Rouen France

Department of Dermatology School of Medicine University of California Davis California

Department of Dermatology School of Medicine University of Utah Salt Lake City Utah

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

Department of Dermatology Stanford University School of Medicine Stanford California; Center for Clinical Sciences Research Palo Alto California; Division of Dermatology Department of Veterans Affairs Palo Alto Healthcare System Palo Alto California

Department of Dermatology University Hospital Marburg Germany

Department of Dermatology University Hospital of Bern Bern Switzerland

Department of Dermatology University of California Irvine California; Department of Biological Chemistry Cancer Center University of California Irvine California; Research Institute Institute for Immunology University of California Irvine California

Department of Dermatology University of Lubeck Lubeck Germany

Department of Dermatology University of North Carolina at Chapel Hill Chapel Hill North Carolina

Department of Dermatovenereology St Anna University Hospital Masaryk University Brno Czech Republic

Department of Immunodermatology Istituto Dermopatico dell'Immacolata IRCCS Rome Italy; Laboratory of Molecular and Cell Biology Istituto Dermopatico dell'Immacolata IRCCS Rome Italy

Dermatology Department School of Medicine Karadeniz Technical University Trabzon Turkey

Dermatology Duke University Medical Center Durham

Division of Molecular Dermatology Department of Dermatology Paracelsus Medical University Salzburg Salzburg Austria

Facultad de Medicina Clínica Alemana Santiago Chile

Kurume University Institute of Cutaneous Cell Biology Kurume Japan

Sheba Medical Center Dermatology Department Tel Hashomer Ramat Gan Israel

St John's Institute of Dermatology Guy's and St Thomas' Hospitals London United Kingdom

St Joseph Mercy Health System Department of Dermatology Ann Arbor Michigan

University Hospitals Leicester Leicester Royal Infirmary Leicester United Kingdom

University Medical Center Groningen University of Groningen Groningen The Netherlands

J Am Acad Dermatol. 2021 Jul;85(1):e47-e48. doi: 10.1016/j.jaad.2021.03.018. PubMed

J Am Acad Dermatol. 2021 Jul;85(1):e45-e46. doi: 10.1016/j.jaad.2021.01.105. PubMed

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