Diagnosis and Management of Pemphigus: recommendations by an International Panel of Experts.

Published online

Journal Article

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 recommendations. METHODS: A preliminary survey, based on the European Dermatology Forum (EDF) and the European Academy of Dermatology and Venereology (EADV) 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 (AAD) conference. A second survey was sent following the meeting to more experts to achieve greater international consensus. RESULTS: The 39 experts participated in the first round of the Delphi-survey while 54 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 based on 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.

Full Text

Duke Authors

Cited Authors

  • Murrell, DF; Peña, S; Joly, P; Marinovic, B; Hashimoto, T; Diaz, LA; Sinha, AA; Payne, AS; Daneshpazhooh, M; Eming, R; Jonkman, MF; Mimouni, D; Borradori, L; Kim, S-C; Yamagami, J; Lehman, JS; Saleh, MA; Culton, DA; Czernik, A; Zone, JJ; Fivenson, D; Ujiie, H; Wozniak, K; Akman-KarakaƟ, A; Bernard, P; Korman, NJ; Caux, F; Drenovska, K; Prost-Squarcioni, C; Vassileva, S; Feldman, RJ; Cardones, AR; Bauer, J; Ioannides, D; Jedlickova, H; Palisson, F; Patsatsi, A; Uzun, S; Yayli, S; Zillikens, D; Amagai, M; Hertl, M; Schmidt, E; Aoki, V; Grando, SA; Shimizu, H; Baum, S; Cianchini, G; Feliciani, C; Iranzo, P; Mascaró, JM; Kowalewski, C; Hall, R; Groves, R; Harman, KE; Marinkovich, MP; Maverakis, E; Werth, VP

Published Date

  • February 10, 2018

Published In

PubMed ID

  • 29438767

Pubmed Central ID

  • 29438767

Electronic International Standard Serial Number (EISSN)

  • 1097-6787

Digital Object Identifier (DOI)

  • 10.1016/j.jaad.2018.02.021


  • eng

Conference Location

  • United States