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Diagnosis and management of pemphigus: Recommendations of an international panel of experts - 11/02/20

Doi : 10.1016/j.jaad.2018.02.021 
Dedee F. Murrell, MA, BMBCh, MD a, , Sandra Peña, MD b, c, Pascal Joly, MD, PhD d, Branka Marinovic, MD, PhD e, Takashi Hashimoto, MD, PhD f, Luis A. Diaz, MD g, Animesh A. Sinha, MD, PhD h, Aimee S. Payne, MD, PhD c, Maryam Daneshpazhooh, MD i, Rüdiger Eming, MD j, Marcel F. Jonkman, MD, PhD k, Daniel Mimouni, MD l, m, Luca Borradori, MD n, Soo-Chan Kim, MD, PhD o, Jun Yamagami, MD, PhD p, Julia S. Lehman, MD q, Marwah Adly Saleh, MD, PhD r, Donna A. Culton, MD, PhD g, Annette Czernik, MD s, John J. Zone, MD t, David Fivenson, MD u, Hideyuki Ujiie, MD, PhD v, Katarzyna Wozniak, MD, PhD w, Ayşe Akman-Karakaş, MD x, Philippe Bernard, MD, PhD y, Neil J. Korman, MD, PhD z, Frédéric Caux, MD, PhD aa, Kossara Drenovska, MD, PhD bb, Catherine Prost-Squarcioni, MD, PhD cc, Snejina Vassileva, MD, PhD bb, Ron J. Feldman, MD, PhD dd, Adela Rambi Cardones, MD ee, Johann Bauer, MD ff, Dimitrios Ioannides, MD, PhD gg, Hana Jedlickova, MD, PhD hh, Francis Palisson, MD ii, Aikaterini Patsatsi, MD, PhD jj, Soner Uzun, MD x, Savas Yayli, MD kk, Detlef Zillikens, MD ll, Masayuki Amagai, MD, PhD p, Michael Hertl, MD mm, Enno Schmidt, MD, PhD ll, Valeria Aoki, MD, PhD nn, Sergei A. Grando, MD, PhD, DSc oo, pp, qq, Hiroshi Shimizu, MD, PhD v, Sharon Baum, MD rr, Guiseppe Cianchini, MD ss, tt, Claudio Feliciani, MD uu, Pilar Iranzo, MD vv, Jose M. Mascaró, MD vv, Cezary Kowalewski, MD w, Russell Hall, MD ee, Richard Groves, MD ww, Karen E. Harman, MB, BChir, DM www, M. Peter Marinkovich, MD xx, yy, zz, Emanual Maverakis, MD aaa, Victoria P. Werth, MD b, c,
a Department of Dermatology, St. George Hospital, University of New South Wales, Sydney, Australia 
b Corporal Michael J. Crescenz Veterans Affairs Medical Center, Philadelphia, Pennsylvania 
c Department of Dermatology at the Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania 
d Department of Dermatology, Rouen University Hospital, Rouen, France 
e Department of Dermatology and Venereology, Zagreb University Hospital Center and School of Medicine, Zagreb, Croatia 
f Kurume University Institute of Cutaneous Cell Biology, Kurume, Japan 
g Department of Dermatology, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina 
h Department of Dermatology, Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, Buffalo, New York 
i Department of Dermatology, Autoimmune Bullous Diseases Research Center, Tehran University of Medical Sciences, Tehran, Iran 
j Department of Dermatology and Allergology, University Hospital, Philipps-Universität Marburg, Marburg, Germany 
k University Medical Center Groningen, University of Groningen, Groningen, The Netherlands 
l Department of Dermatology, Rabin Medical Center, Beilinson Campus, Petach Tikva, Israel 
m Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel 
n Department of Dermatology, University Hospital of Bern, Bern, Switzerland 
o Department of Dermatology, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul, Korea 
p Department of Dermatology, Keio University School of Medicine, Tokyo, Japan 
q Department of Dermatology, Mayo Clinic College of Medicine, Rochester, Minnesota 
r Department of Dermatology, Faculty of Medicine, Cairo University, Cairo, Egypt 
s Department of Dermatology, Icahn School of Medicine at Mount Sinai, New York, New York 
t Department of Dermatology, School of Medicine, University of Utah, Salt Lake City, Utah 
u St. Joseph Mercy Health System, Department of Dermatology, Ann Arbor, Michigan 
v Department of Dermatology, Hokkaido University Graduate School of Medicine, Sapporo, Japan 
w Department of Dermatology and Immunodermatology, Medical University of Warsaw, Warsaw, Poland 
x Department of Dermatology and Venereology, Faculty of Medicine, Akdeniz University, Antalya, Turkey 
y Department of Dermatology, Reims University Hospital, University of Champagne-Ardenne, Reims, France 
z Department of Dermatology and the Murdough Family Center for Psoriasis, University Hospitals Case Medical Center, Cleveland, Ohio 
aa Department of Dermatology, Avicenne Hospital, University Paris 13, Bobigny, France 
cc Department of Dermatology, Department of Histology, Reference Center for Autoimmune Bullous Diseases, Avicenne Hospital, University Paris 13, Bobigny, France 
bb Department of Dermatology and Venereology, Medical Faculty, University of Medicine, Sofia, Bulgaria 
dd Department of Dermatology, Emory University School of Medicine, Atlanta, Georgia 
ee Dermatology, Duke University Medical Center, Durham 
ff Division of Molecular Dermatology, Department of Dermatology, Paracelsus Medical University Salzburg, Salzburg, Austria 
gg First Department of Dermatology, Aristotle University, Thessaloniki, Greece 
hh Department of Dermatovenereology, St. Anna University Hospital, Masaryk University, Brno, Czech Republic 
ii Facultad de Medicina, Clínica Alemana, Santiago, Chile 
jj Second Dermatology Department, Aristotle University School of Medicine, Papageorgiou General Hospital, Thessaloniki, Greece 
kk Dermatology Department, School of Medicine, Karadeniz Technical University, Trabzon, Turkey 
ll Department of Dermatology, University of Lubeck, Lubeck, Germany 
mm Department of Dermatology, University Hospital, Marburg, Germany 
nn Departamento de Dermatologia, Universidade de Sao Paulo, Sao Paulo, Brazil 
oo Department of Dermatology, University of California, Irvine, California 
pp Department of Biological Chemistry Cancer Center, University of California, Irvine, California 
qq Research Institute, Institute for Immunology, University of California, Irvine, California 
rr Sheba Medical Center, Dermatology Department, Tel-Hashomer, Ramat-Gan, Israel 
ss Department of Immunodermatology, Istituto Dermopatico dell'Immacolata, IRCCS, Rome, Italy 
tt Laboratory of Molecular and Cell Biology, Istituto Dermopatico dell'Immacolata, IRCCS, Rome, Italy 
uu Clinica Dermatologica, Universita' Di Parma, Parma, Italy 
vv Department of Dermatology, Hospital Clínic, Universitat de Barcelona, Barcelona, Spain 
ww St. John's Institute of Dermatology, Guy's & St. Thomas' Hospitals, London, United Kingdom 
www University Hospitals Leicester, Leicester Royal Infirmary, Leicester, United Kingdom 
xx Department of Dermatology, Stanford University School of Medicine, Stanford, California 
yy Center for Clinical Sciences Research, Palo Alto, California 
zz Division of Dermatology, Department of Veterans Affairs Palo Alto Healthcare System, Palo Alto, California 
aaa Department of Dermatology, School of Medicine, University of California, Davis, California 

Correspondence to: Dedee F. Murrell, MA, MD, FACD, Department of Dermatology, St. George Hospital, Gray Street, Kogarah, Sydney, NSW 2217, Australia.Department of DermatologySt. George HospitalGray Street, KogarahSydneyNSW2217Australia∗∗Victoria P. Werth, MD, Department of Dermatology, University of Pennsylvania, Philadelphia, PA.Department of DermatologyUniversity of PennsylvaniaPhiladelphiaPA



Several European countries recently developed international diagnostic and management guidelines for pemphigus, which have been instrumental in the standardization of pemphigus management.


We now present results from a subsequent Delphi consensus to broaden the generalizability of the recommendations.


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.


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.


Each recommendation represents the majority opinion and therefore may not reflect all possible treatment options available.


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.

Le texte complet de cet article est disponible en PDF.

Key words : CD20 inhibitor, consensus, guidelines, pemphigus foliaceus, pemphigus vulgaris, treatment

Abbreviations used : ABSIS, DIF, Dsg1, Dsg3, ELISA, IIF, PDAI


 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.
 Reprints not available from the authors.

© 2020  Publié par Elsevier Masson SAS.
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Vol 82 - N° 3

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  • Shanthi Narla, Jonathan I. Silverberg

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