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Diagnosis and management of pemphigus: Recommendations of an international panel of experts.
Journal of the American Academy of Dermatology ( IF 12.8 ) Pub Date : 2018-02-10 , DOI: 10.1016/j.jaad.2018.02.021
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 44 , Cezary Kowalewski 22 , Russell Hall 30 , Richard Groves 45 , Karen E Harman 46 , M Peter Marinkovich 47 , Emanual Maverakis 48 , Victoria P Werth 2
Affiliation  

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.

中文翻译:

天疱疮的诊断和管理:国际专​​家小组的建议。

背景 几个欧洲国家最近制定了天疱疮的国际诊断和管理指南,这些指南有助于天疱疮管理的标准化。目标我们现在展示来自随后的德尔菲共识的结果,以扩大建议的普遍性。方法 根据欧洲皮肤病学论坛和欧洲皮肤病学和性病学会指南,将初步调查发送给国际专家小组以确定共识水平。2016 年 3 月,在美国皮肤病学会年度会议期间,国际大疱病共识小组讨论了该结果。会后,向更多专家发送了第二次调查,以达成更大的国际共识。结果 39位专家参加了第一轮德尔福调查,来自21个国家的54位专家完成了第二轮。根据Delphi结果和会议讨论,调查中的陈述数量从Delphi I的175个主题减少到Delphi II的24个主题。局限性 每条建议都代表多数意见,因此可能无法反映所有可能的治疗方案。结论 我们在此提出了从 Delphi 过程中得出的建议。该国际共识包括静脉注射 CD20 抑制剂作为中重度天疱疮的一线治疗选择。根据Delphi结果和会议讨论,调查中的陈述数量从Delphi I的175个主题减少到Delphi II的24个主题。局限性 每条建议都代表多数意见,因此可能无法反映所有可能的治疗方案。结论 我们在此提出了从这个 Delphi 过程中得出的建议。该国际共识包括静脉注射 CD20 抑制剂作为中重度天疱疮的一线治疗选择。根据Delphi结果和会议讨论,调查中的陈述数量从Delphi I的175个主题减少到Delphi II的24个主题。局限性 每条建议都代表多数意见,因此可能无法反映所有可能的治疗方案。结论 我们在此提出了从 Delphi 过程中得出的建议。该国际共识包括静脉注射 CD20 抑制剂作为中重度天疱疮的一线治疗选择。
更新日期:2020-02-20
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