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Diagnosis and management of pemphigus: Recommendations of an international panel of experts
DF. Murrell, S. Peña, P. Joly, B. Marinovic, T. Hashimoto, LA. Diaz, AA. Sinha, AS. Payne, M. Daneshpazhooh, R. Eming, MF. Jonkman, D. Mimouni, L. Borradori, SC. Kim, J. Yamagami, JS. Lehman, MA. Saleh, DA. Culton, A. Czernik, JJ. Zone, D....
Language English Country United States
Document type Journal Article
Grant support
P30 AR069589
NIAMS NIH HHS - United States
R01 AR032599
NIAMS NIH HHS - United States
T32 AR007465
NIAMS NIH HHS - United States
- MeSH
- Academies and Institutes standards MeSH
- Antigens, CD20 immunology MeSH
- Delphi Technique MeSH
- Dermatology methods standards MeSH
- Glucocorticoids administration & dosage MeSH
- Immunologic Factors administration & dosage MeSH
- Administration, Intravenous MeSH
- Drug Therapy, Combination methods standards MeSH
- Combined Modality Therapy methods standards MeSH
- Consensus MeSH
- Humans MeSH
- Pemphigus diagnosis immunology therapy MeSH
- Plasmapheresis * MeSH
- Rituximab administration & dosage MeSH
- Practice Guidelines as Topic * MeSH
- Severity of Illness Index MeSH
- Check Tag
- Humans MeSH
- Publication type
- Journal Article MeSH
- Geographicals
- Europe MeSH
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
Center for Clinical Sciences Research Palo Alto California
Clinica Dermatologica Universita' Di Parma Parma Italy
Corporal Michael J Crescenz Veterans Affairs Medical Center Philadelphia Pennsylvania
Departamento de Dermatologia Universidade de Sao Paulo Sao Paulo Brazil
Department of Biological Chemistry Cancer Center University of California Irvine California
Department of Dermatology and Immunodermatology Medical University of Warsaw Warsaw Poland
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 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 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 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
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
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 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
Dermatology Department School of Medicine Karadeniz Technical University Trabzon Turkey
Dermatology Duke University Medical Center Durham
Facultad de Medicina Clínica Alemana Santiago Chile
Kurume University Institute of Cutaneous Cell Biology Kurume Japan
Laboratory of Molecular and Cell Biology Istituto Dermopatico dell'Immacolata IRCCS Rome Italy
Research Institute Institute for Immunology University of California Irvine California
Sackler School of Medicine Tel Aviv University Tel Aviv Israel
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
References provided by Crossref.org
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- $a Murrell, Dedee F $u Department of Dermatology, St. George Hospital, University of New South Wales, Sydney, Australia. Electronic address: d.murrell@unsw.edu.au.
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- $a Diagnosis and management of pemphigus: Recommendations of an international panel of experts / $c DF. Murrell, S. Peña, P. Joly, B. Marinovic, T. Hashimoto, LA. Diaz, AA. Sinha, AS. Payne, M. Daneshpazhooh, R. Eming, MF. Jonkman, D. Mimouni, L. Borradori, SC. Kim, J. Yamagami, JS. Lehman, MA. Saleh, DA. Culton, A. Czernik, JJ. Zone, D. Fivenson, H. Ujiie, K. Wozniak, A. Akman-Karakaş, P. Bernard, NJ. Korman, F. Caux, K. Drenovska, C. Prost-Squarcioni, S. Vassileva, RJ. Feldman, AR. Cardones, J. Bauer, D. Ioannides, H. Jedlickova, F. Palisson, A. Patsatsi, S. Uzun, S. Yayli, D. Zillikens, M. Amagai, M. Hertl, E. Schmidt, V. Aoki, SA. Grando, H. Shimizu, S. Baum, G. Cianchini, C. Feliciani, P. Iranzo, JM. Mascaró, C. Kowalewski, R. Hall, R. Groves, KE. Harman, MP. Marinkovich, E. Maverakis, VP. Werth,
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- $a 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.
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