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

Dedee F. Murrell, Sandra Peña, Pascal Joly, Branka Marinovic, Takashi Hashimoto, Luis A. Diaz, Animesh A. Sinha, Aimee S. Payne, Maryam Daneshpazhooh, Rüdiger Eming, Marcel F. Jonkman, Daniel Mimouni, Luca Borradori, Soo Chan Kim, Jun Yamagami, Julia S. Lehman, Marwah Adly Saleh, Donna A. Culton, Annette Czernik, John J. ZoneDavid Fivenson, Hideyuki Ujiie, Katarzyna Wozniak, Ayşe Akman-Karakaş, Philippe Bernard, Neil J. Korman, Frédéric Caux, Kossara Drenovska, Catherine Prost-Squarcioni, Snejina Vassileva, Ron J. Feldman, Adela Rambi Cardones, Johann Bauer, Dimitrios Ioannides, Hana Jedlickova, Francis Palisson, Aikaterini Patsatsi, Soner Uzun, Savas Yayli, Detlef Zillikens, Masayuki Amagai, Michael Hertl, Enno Schmidt, Valeria Aoki, Sergei A. Grando, Hiroshi Shimizu, Sharon Baum, Guiseppe Cianchini, Claudio Feliciani, Pilar Iranzo, Jose M. Mascaró, Cezary Kowalewski, Russell Hall, Richard Groves, Karen E. Harman, M. Peter Marinkovich, Emanual Maverakis, Victoria P. Werth

Research output: Contribution to journalArticlepeer-review

133 Citations (Scopus)


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.

Original languageEnglish
Pages (from-to)575-585.e1
JournalJournal of the American Academy of Dermatology
Issue number3
Publication statusPublished - 2020 Mar

Bibliographical note

Funding Information:
Funding sources: 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. Disclosure: Dr Murrell is an investigator and speaker for Roche; she is an investigator for and on an advisory board for Principia Biopharma and is also on an advisory board for Immune Pharmaceuticals and Lilly. Dr Joly is a consultant for Roche, Principia Biopharma, Lillyand Biogen. Dr Payne is a consultant for Syntimmune and TG Therapeutics and receives grants Sanofi. Dr Eming is an investigator for both Biotest AG and Fresenius Medical Care and is a speaker for Biotest AG and Novartis. Dr Jonkman is a monitor for Roche. Dr Prost is an investigator for Roche. Dr Yayli is an investigator for Roche. Dr Zillikens is on the advisory board for Roche and a consultant for Euroimmun, UCB, Fresenius, Almirall, and oGEN-x. Dr Zillikens has received grants and is a speaker for Euroimmun, Miltenyi/Biogen, Fresenius/Roche, Biotest AG, Almirall, and Dompe/Janssen. Dr Amagai receives speaker honoraria and grants from Nihon Pharmaceutical and research support from Medical & Biological Laboratories. Dr Hertl is on an advisory boards for Roche, Biogen, and Novartis; he has received grants from Biotest and Fresenius and is a speaker for Janssen. Dr Schmidt has received grants from Euroimmun and Fresenius and is a speaker for Biotest and Fresenius. Dr Hall is a consultant for Stieffel at GlaxoSmithKline Company, Eli Lilly, Syntimmune, and Immune Sciences and is on the data safety monitoring board for Roche and has received grants from Immune Sciences. Dr Peña, Dr Marinovic, Dr Hashimoto, Dr Diaz, Dr Sinha, Dr Daneshpazhooh, Dr Mimouni, Dr Borradori, Dr Kim, Dr Yamagami, Dr Lehman, Dr Saleh, Dr Culton, Dr Czernik, Dr Zone, Dr Fivenson, Dr Ujiie, Dr Wozniak, Dr Akman-Karakaş, Dr Bernard, Dr Korman, Dr Caux, Dr Drenovska, Dr Vassileva, Dr Feldman, Dr Cardones, Dr Bauer, Dr Ioannides, Dr Jedlickova, Dr Palisson, Dr Patsatsi, Dr Uzun, Dr Aoki, Dr Grando, Dr Shimizu, Dr Baum, Dr Cianchini, Dr Feliciani, Dr Iranzo, Dr Mascaró Jr, Dr Kowalewski, Dr Groves, Dr Harman, Dr Marinkovich, Dr Maverakis, and Dr Werth have no conflicts of interest to disclose.

Publisher Copyright:
© 2020

All Science Journal Classification (ASJC) codes

  • Dermatology


Dive into the research topics of 'Diagnosis and management of pemphigus: Recommendations of an international panel of experts'. Together they form a unique fingerprint.

Cite this