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Updated S2k Guidelines on the Management of Pemphigus Vulgaris and Foliaceus Initiated by the European Academy of Dermatology and Venereology (Eadv) Publisher Pubmed



Joly P1 ; Horvath B2 ; Patsatsi 3 ; Uzun S4 ; Bech R5 ; Beissert S6 ; Bergman R7 ; Bernard P8 ; Borradori L9 ; Caproni M10 ; Caux F11 ; Cianchini G12 ; Daneshpazhooh M13 ; De D14 Show All Authors
Authors
  1. Joly P1
  2. Horvath B2
  3. Patsatsi 3
  4. Uzun S4
  5. Bech R5
  6. Beissert S6
  7. Bergman R7
  8. Bernard P8
  9. Borradori L9
  10. Caproni M10
  11. Caux F11
  12. Cianchini G12
  13. Daneshpazhooh M13
  14. De D14
  15. Dmochowski M15
  16. Drenovska K16
  17. Ehrchen J17
  18. Feliciani C18
  19. Goebeler M19
  20. Groves R20
  21. Guenther C21
  22. Hofmann S22
  23. Ioannides D23
  24. Kowalewski C24
  25. Ludwig R21
  26. Lim YL25
  27. Marinovic B26
  28. Marzano AV27, 28
  29. Mimouni D30
  30. Murrell DF31
  31. Pincelli C32
  32. Squarcioni CP11
  33. Sardy M33
  34. Setterfield J34
  35. Sprecher E36, 37
  36. Vassileva S16
  37. Wozniak K38
  38. Yayli S39
  39. Zambruno G40
  40. Zillikens D41
  41. Hertl M42
  42. Schmidt E41
Show Affiliations
Authors Affiliations
  1. 1. Department of Dermatology, Rouen University Hospital and INSERM U905, Centre de reference des maladies bulleuses autoimmunes, Normandie University, Rouen, France
  2. 2. Department of Dermatology, University Medical Center Groningen, Center for Blistering Diseases, University of Groningen, Groningen, Netherlands
  3. 3. Autoimmune Bullous Diseases Unit, 2nd Dermatology Department, Papageorgiou General Hospital, Αristotle University School of Medicine, Thessaloniki, Greece
  4. 4. Department of Dermatology, Akdeniz University Faculty of Medicine, Antalya, Turkey
  5. 5. Department of Dermatology, Aarhus University Hospital, Aarhus, Denmark
  6. 6. Department of Dermatology, University Hospital Carl Gustav Carus, Dresden, Germany
  7. 7. Department of Dermatology, Rambam Health Care Campus and The Ruth and Bruce Rappaport, Faculty of Medicine, Haifa, Israel
  8. 8. University of Reims-Champagne-Ardenne, Reims, France
  9. 9. Department of Dermatology, University Hospital of Bern, University of Bern, Bern, Switzerland
  10. 10. Department of Health Sciences, Section of Dermatology, USL Toscana Centro, Rare Diseases Unit, European Reference Network-Skin Member, University of Florence, Florence, Italy
  11. 11. Department of Dermatology and Referral Center for Autoimmune Bullous Diseases MALIBUL, Avicenne Hospital, AP-HP, University Paris 13, Bobigny, France
  12. 12. Department of Dermatology, Cristo Re Hospital, Rome, Italy
  13. 13. Autoimmune Bullous Diseases Research Center, Department of Dermatology, Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran
  14. 14. Department of Dermatology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
  15. 15. Autoimmune Blistering Dermatoses Section, Department of Dermatology, Poznan University of Medical Sciences, Poznan, Poland
  16. 16. Department of Dermatology, Medical University, Sofia, Bulgaria
  17. 17. Department of Dermatology, University of Munster, Munster, Germany
  18. 18. Dermatology unit, Azienda ospedaliero -universitaria, Universita di Parma, Parma, Italy
  19. 19. Department of Dermatology, Venereology and Allergology, University Hospital Wurzburg, Wurzburg, Germany
  20. 20. St John's Institute of Dermatology, London, United Kingdom
  21. 21. Department of Dermatology, University Hospital, Technical University Dresden, Dresden, Germany
  22. 22. Department of Dermatology, Allergy and Dermatosurgery, Helios University Hospital Wuppertal, University Witten, Herdecke, Germany
  23. 23. 1st Department of Dermatology-Venereology, Hospital of Skin and Venereal Diseases, Aristotle University Medical School, Thessaloniki, Greece
  24. 24. Department Dermatology and Immunodermatology, Medical University of Warsaw, Warsaw, Poland
  25. 25. National Skin Centre, Singapore, Singapore
  26. 26. Department of Dermatology and Venereology, University Hospital Centre Zagreb, School of Medicine, University of Zagreb, Zagreb, Croatia
  27. 27. Dermatology Unit, Fondazione IRCCS Ca Granda Ospedale Maggiore Policlinico, Milan, Italy
  28. 28. Department of Physiopathology and Transplantation, Universita degli Studi di Milano, Milan, Italy
  29. 29. Department of Dermatology, Hospital Clinic de Barcelona, Universitat de Barcelona, Barcelona, Spain
  30. 30. Department of Dermatology, Rabin Medical Center, petha-Tikwa and Sackler School of Medicine, Tel Aviv University, Tel aviv, Israel
  31. 31. Department of Dermatology, St George Hospital, University of NSW, Sydney, NSW, Australia
  32. 32. DermoLab, Institute of Dermatology, University of Modena and Reggio Emilia, Modena, Italy
  33. 33. Department of Dermatology, Venereology, and Dermatooncology, Semmelweis University, Budapest, Hungary
  34. 34. Department of Oral Medicine, St John's Institute of Dermatology
  35. 35. Guy's and St Thomas' NHS Foundation Trust, London, United Kingdom
  36. 36. Division of Dermatology, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel
  37. 37. Department of Human Molecular Genetics and Biochemistry, Sackler Faculty of Medicine, Tel-Aviv University, Tel Aviv, Israel
  38. 38. Department of Dermatology and Immunodermatology, Medical University of Warsaw, Warsaw, Poland
  39. 39. Department of Dermatology, Karadeniz Technical University Faculty of Medicine, Trabzon, Turkey
  40. 40. Genetics and rare Disease Research Division, Bambino Gesu Children’s Hospital, IRCCS, Rome, Italy
  41. 41. Department of Dermatology and Lubeck Institute of Experimental Dermatology (LIED), University of Lubeck, Lubeck, Germany
  42. 42. Department of Dermatology, University of Marburg, Marburg, Germany

Source: Journal of the European Academy of Dermatology and Venereology Published:2020


Abstract

Background: Pemphigus encompasses a group of life-threatening autoimmune bullous diseases characterized by blisters and erosions of the mucous membranes and skin. Before the era of immunosuppressive treatment, pemphigus was almost always fatal. Due to its rarity, only few randomized controlled therapeutic trials are available. Recently, rituximab has been approved as first-line treatment for moderate and severe pemphigus vulgaris in Europe and the United States. Objectives: The Autoimmune blistering diseases Task Force of the European Academy of Dermatology and Venereology (EADV) has initiated a throughout update of the guideline for the management of patients with pemphigus. Results: The guidelines for the management of pemphigus were updated, and the degree of consent among all task force members was included. The final version of the guideline was consented by the European Dermatology Forum (EDF) and several patient organizations. © 2020 European Academy of Dermatology and Venereology
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