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Diagnosis and Management of Pemphigus: Recommendations of an International Panel of Experts Publisher Pubmed



Murrell DF1 ; Pena S2, 3 ; Joly P4 ; Marinovic B5 ; Hashimoto T6 ; Diaz LA7 ; Sinha AA8 ; Payne AS3 ; Daneshpazhooh M9 ; Eming R10 ; Jonkman MF11 ; Mimouni D12, 13 ; Borradori L14 ; Kim SC15 Show All Authors
Authors
  1. Murrell DF1
  2. Pena S2, 3
  3. Joly P4
  4. Marinovic B5
  5. Hashimoto T6
  6. Diaz LA7
  7. Sinha AA8
  8. Payne AS3
  9. Daneshpazhooh M9
  10. Eming R10
  11. Jonkman MF11
  12. Mimouni D12, 13
  13. Borradori L14
  14. Kim SC15
  15. Yamagami J16
  16. Lehman JS17
  17. Saleh MA18
  18. Culton DA7
  19. Czernik A19
  20. Zone JJ20
  21. Fivenson D21
  22. Ujiie H22
  23. Wozniak K23
  24. Akmankarakas A24
  25. Bernard P25
  26. Korman NJ26
  27. Caux F27
  28. Drenovska K29
  29. Prostsquarcioni C28
  30. Vassileva S29
  31. Feldman RJ30
  32. Cardones AR31
  33. Bauer J32
  34. Ioannides D33
  35. Jedlickova H34
  36. Palisson F35
  37. Patsatsi A36
  38. Uzun S24
  39. Yayli S37
  40. Zillikens D38
  41. Amagai M16
  42. Hertl M39
  43. Schmidt E38
  44. Aoki V40
  45. Grando SA41, 42, 43
  46. Shimizu H22
  47. Baum S44
  48. Cianchini G45, 46
  49. Feliciani C47
  50. Iranzo P48
  51. Kowalewski C23
  52. Hall R31
  53. Groves R49
  54. Harman KE50
  55. Marinkovich MP51, 52, 53
  56. Maverakis E54
  57. Werth VP2, 3
Show Affiliations
Authors Affiliations
  1. 1. Department of Dermatology, St. George Hospital, University of New South Wales, Sydney, Australia
  2. 2. Corporal Michael J. Crescenz Veterans Affairs Medical Center, Philadelphia, PA, United States
  3. 3. Department of Dermatology at the Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, United States
  4. 4. Department of Dermatology, Rouen University Hospital, Rouen, France
  5. 5. Department of Dermatology and Venereology, Zagreb University Hospital Center and School of Medicine, Zagreb, Croatia
  6. 6. Kurume University Institute of Cutaneous Cell Biology, Kurume, Japan
  7. 7. Department of Dermatology, University of North Carolina at Chapel Hill, Chapel Hill, NC, United States
  8. 8. Department of Dermatology, Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, Buffalo, NY, United States
  9. 9. Department of Dermatology, Autoimmune Bullous Diseases Research Center, Tehran University of Medical Sciences, Tehran, Iran
  10. 10. Department of Dermatology and Allergology, University Hospital, Philipps-Universitat Marburg, Marburg, Germany
  11. 11. University Medical Center Groningen, University of Groningen, Groningen, Netherlands
  12. 12. Department of Dermatology, Rabin Medical Center, Beilinson Campus, Petach Tikva, Israel
  13. 13. Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel
  14. 14. Department of Dermatology, University Hospital of Bern, Bern, Switzerland
  15. 15. Department of Dermatology, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul, South Korea
  16. 16. Department of Dermatology, Keio University School of Medicine, Tokyo, Japan
  17. 17. Department of Dermatology, Mayo Clinic College of Medicine, Rochester, MN, United States
  18. 18. Department of Dermatology, Faculty of Medicine, Cairo University, Cairo, Egypt
  19. 19. Department of Dermatology, Icahn School of Medicine at Mount Sinai, New York, NY, United States
  20. 20. Department of Dermatology, School of Medicine, University of Utah, Salt Lake City, UT, United States
  21. 21. St. Joseph Mercy Health System, Department of Dermatology, Ann Arbor, MI, United States
  22. 22. Department of Dermatology, Hokkaido University Graduate School of Medicine, Sapporo, Japan
  23. 23. Department of Dermatology and Immunodermatology, Medical University of Warsaw, Warsaw, Poland
  24. 24. Department of Dermatology and Venereology, Faculty of Medicine, Akdeniz University, Antalya, Turkey
  25. 25. Department of Dermatology, Reims University Hospital, University of Champagne-Ardenne, Reims, France
  26. 26. Department of Dermatology and the Murdough Family Center for Psoriasis, University Hospitals Case Medical Center, Cleveland, OH, United States
  27. 27. Department of Dermatology, Avicenne Hospital, University Paris 13, Bobigny, France
  28. 28. Department of Dermatology, Department of Histology, Reference Center for Autoimmune Bullous Diseases, Avicenne Hospital, University Paris 13, Bobigny, France
  29. 29. Department of Dermatology and Venereology, Medical Faculty, University of Medicine, Sofia, Bulgaria
  30. 30. Department of Dermatology, Emory University School of Medicine, Atlanta, Georgia, Georgia
  31. 31. Dermatology, Duke University Medical Center, Durham, United Kingdom
  32. 32. Division of Molecular Dermatology, Department of Dermatology, Paracelsus Medical University Salzburg, Salzburg, Austria
  33. 33. First Department of Dermatology, Aristotle University, Thessaloniki, Greece
  34. 34. Department of Dermatovenereology, St. Anna University Hospital, Masaryk University, Brno, Czech Republic
  35. 35. Facultad de Medicina, Clinica Alemana, Santiago, Chile
  36. 36. Second Dermatology Department, Aristotle University School of Medicine, Papageorgiou General Hospital, Thessaloniki, Greece
  37. 37. Dermatology Department, School of Medicine, Karadeniz Technical University, Trabzon, Turkey
  38. 38. Department of Dermatology, University of Lubeck, Lubeck, Germany
  39. 39. Department of Dermatology, University Hospital, Marburg, Germany
  40. 40. Departamento de Dermatologia, Universidade de Sao Paulo, Sao Paulo, Brazil
  41. 41. Department of Dermatology, University of California, Irvine, CA, United States
  42. 42. Department of Biological Chemistry Cancer Center, University of California, Irvine, CA, United States
  43. 43. Research Institute, Institute for Immunology, University of California, Irvine, CA, United States
  44. 44. Sheba Medical Center, Dermatology Department, Tel-Hashomer, Ramat-Gan, Israel
  45. 45. Department of Immunodermatology, Istituto Dermopatico dell'Immacolata, IRCCS, Rome, Italy
  46. 46. Laboratory of Molecular and Cell Biology, Istituto Dermopatico dell'Immacolata, IRCCS, Rome, Italy
  47. 47. Clinica Dermatologica, Universita' Di Parma, Parma, Italy
  48. 48. Department of Dermatology, Hospital Clinic, Universitat de Barcelona, Barcelona, Spain
  49. 49. St. John's Institute of Dermatology, Guy's & St. Thomas' Hospitals, London, United Kingdom
  50. 50. University Hospitals Leicester, Leicester Royal Infirmary, Leicester, United Kingdom, United Kingdom
  51. 51. Department of Dermatology, Stanford University School of Medicine, Stanford, CA, United States
  52. 52. Center for Clinical Sciences Research, Palo Alto, CA, United States
  53. 53. Division of Dermatology, Department of Veterans Affairs Palo Alto Healthcare System, Palo Alto, CA, United States
  54. 54. Department of Dermatology, School of Medicine, University of California, Davis, CA, United States

Source: Journal of the American Academy of Dermatology Published:2020


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. © 2020