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. 2020 Mar;82(3):575-585.e1.
doi: 10.1016/j.jaad.2018.02.021. Epub 2018 Feb 10.

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

Dedee F Murrell  1 , Sandra Peña  2 , Pascal Joly  3 , Branka Marinovic  4 , Takashi Hashimoto  5 , Luis A Diaz  6 , Animesh A Sinha  7 , Aimee S Payne  8 , Maryam Daneshpazhooh  9 , Rüdiger Eming  10 , Marcel F Jonkman  11 , Daniel Mimouni  12 , Luca Borradori  13 , Soo-Chan Kim  14 , Jun Yamagami  15 , Julia S Lehman  16 , Marwah Adly Saleh  17 , Donna A Culton  6 , Annette Czernik  18 , John J Zone  19 , David Fivenson  20 , Hideyuki Ujiie  21 , Katarzyna Wozniak  22 , Ayşe Akman-Karakaş  23 , Philippe Bernard  24 , Neil J Korman  25 , Frédéric Caux  26 , Kossara Drenovska  27 , Catherine Prost-Squarcioni  28 , Snejina Vassileva  27 , Ron J Feldman  29 , Adela Rambi Cardones  30 , Johann Bauer  31 , Dimitrios Ioannides  32 , Hana Jedlickova  33 , Francis Palisson  34 , Aikaterini Patsatsi  35 , Soner Uzun  23 , Savas Yayli  36 , Detlef Zillikens  37 , Masayuki Amagai  15 , Michael Hertl  38 , Enno Schmidt  37 , Valeria Aoki  39 , Sergei A Grando  40 , Hiroshi Shimizu  21 , Sharon Baum  41 , Guiseppe Cianchini  42 , Claudio Feliciani  43 , Pilar Iranzo  44 , Jose M Mascaró Jr  44 , Cezary Kowalewski  22 , Russell Hall  30 , Richard Groves  45 , Karen E Harman  46 , M Peter Marinkovich  47 , Emanual Maverakis  48 , Victoria P Werth  49
Affiliations

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

Dedee F Murrell et al. J Am Acad Dermatol. 2020 Mar.

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.

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Figures

Fig 1.
Fig 1.
Diagnosis of pemphigus. Diagnosis requires clinical presentation and histopathology that are consistent with pemphigus and either a positive direct immunofluorescence (DIF) microscopy result or serologic detection of autoantibodies against epithelial cell surface antigens. ELISA, Enzyme-linked immunosorbent assay; PF, pemphigus foliaceus; PV, pemphigus vulgaris; WOCBP, women of childbearing potential.
Fig 2.
Fig 2.
Treatment options. The principal objective is to promote the healing of blisters and erosions, prevent development of new lesions, and minimize serious side effects of treatment.
See this image and copyright information in PMC

Comment in

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