Diagnosis and management of pemphigus: Recommendations of an international panel of experts

• Type of Material: Article
• Authors: Dedee F. Murrell, University of New South Wales; Sandra Pena, Corporal Michael J Crescenz Veteran Affairs Med Ctr; Pascal Joly, Rouen University Hospital; Branka Marinovic, Zagreb University; Takashi Hashimoto, Kurume University Institute of Cutaneous Cell Biology; Luis A. Diaz, University of North Carolina; Animesh A. Sinha, University of Buffalo; Aimee S. Payne, University of Pennsylvania; Maryam Daneshpazhooh, Tehran University of Medical Sciences; Ron Feldman, Emory University
• Language: English
• Date: 2020-03-01
• Publisher: Mosby-Elsevier
• Publication Version: Author Accepted Manuscript (After Peer Review)
• Copyright Statement: © 2020 by the American Academy of Dermatology, Inc.
• License: Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International
• Final Published Version (URL): http://dx.doi.org/10.1016/j.jaad.2018.02.021
• Title of Journal or Parent Work: Journal of the American Academy of Dermatology
• Volume: 82
• Issue: 3
• Start Page: 575
• End Page: +
• Grant/Funding Information: Supported in part by the Office of Research and Development, Biomedical Laboratory Research and Development, Veterans Health Administration, US Department of Veterans Affairs. Dr Pena’s clinical research is funded by a National Institutes of Health training grant.
• Keywords: pemphigus vulgaris; CD20 inhibitor; Life Sciences & Biomedicine; treatment; Dermatology; Guidelines; guidelines; consensus; Science & Technology; pemphigus foliaceus

Abstract

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.