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Journal Articles Journal of The American Academy of Dermatology Year : 2020

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

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Dedee F Murrell
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Sandra Peña
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Pascal Joly
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Branka Marinovic
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Takashi Hashimoto
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Luis A Diaz
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Animesh A Sinha
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Aimee S Payne
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Maryam Daneshpazhooh
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Rüdiger Eming
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Marcel F Jonkman
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Daniel Mimouni
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Luca Borradori
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Soo-Chan Kim
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Jun Yamagami
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Julia S Lehman
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Marwah Adly Saleh
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Donna A Culton
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Annette Czernik
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John J Zone
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David Fivenson
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Hideyuki Ujiie
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Katarzyna Wozniak
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Ayşe Akman-Karakaş
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Neil J Korman
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Frédéric Caux
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Kossara Drenovska
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Catherine Prost-Squarcioni
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Snejina Vassileva
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Ron J Feldman
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Adela Rambi Cardones
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Johann Bauer
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Dimitrios Ioannides
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Hana Jedlickova
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Francis Palisson
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Aikaterini Patsatsi
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Soner Uzun
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Savas Yayli
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Detlef Zillikens
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Masayuki Amagai
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Michael Hertl
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Enno Schmidt
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Valeria Aoki
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Sergei A Grando
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Hiroshi Shimizu
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Sharon Baum
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Guiseppe Cianchini
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Claudio Feliciani
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Pilar Iranzo
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Jose M Mascaró
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Cezary Kowalewski
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Russell Hall
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Richard Groves
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Karen E Harman
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M. Peter Peter Marinkovich
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Emanual Maverakis
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Victoria P Werth
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Marwah Adly Saleh
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Adela Rambi Cardones
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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. Keywords CD20 inhibitor; consensus; guidelines; pemphigus foliaceus; pemphigus vulgaris; treatment Pemphigus encompasses a spectrum of rare mucocutaneous bullous diseases that are autoimmune in origin. Because of the rarity of these diseases, it can take patients months before their pemphigus is diagnosed, during which time many are treated for other blistering diseases. 1,2 Even once the diagnosis has been made, treatment regimens can vary greatly, as
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Dates and versions

hal-03434491 , version 1 (18-11-2021)

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Dedee F Murrell, Sandra Peña, Pascal Joly, Branka Marinovic, Takashi Hashimoto, et al.. Diagnosis and management of pemphigus: Recommendations of an international panel of experts. Journal of The American Academy of Dermatology, 2020, 82 (3), pp.575 - 585.e1. ⟨10.1016/j.jaad.2018.02.021⟩. ⟨hal-03434491⟩

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