P Joly1, B Horvath2, Α Patsatsi3, S Uzun4, R Bech5, S Beissert6, R Bergman7, P Bernard8, L Borradori9, M Caproni10, F Caux11, G Cianchini12, M Daneshpazhooh13, D De14, M Dmochowski15, K Drenovska16, J Ehrchen17, C Feliciani18, M Goebeler19, R Groves20, C Guenther21, S Hofmann22, D Ioannides23, C Kowalewski24, R Ludwig21, Y L Lim25, B Marinovic26, A V Marzano27,28, J M Mascaró29, D Mimouni30, D F Murrell31, C Pincelli32, C P Squarcioni11, M Sárdy33, J Setterfield34, E Sprecher35,36, S Vassileva16, K Wozniak37, S Yayli38, G Zambruno39, D Zillikens40, M Hertl41, E Schmidt40. 1. Department of Dermatology, Rouen University Hospital and INSERM U905, Centre de référence des maladies bulleuses autoimmunes, Normandie University, Rouen, France. 2. Department of Dermatology, University Medical Center Groningen, Center for Blistering Diseases, University of Groningen, Groningen, the Netherlands. 3. Autoimmune Bullous Diseases Unit, 2nd Dermatology Department, Papageorgiou General Hospital, Αristotle University School of Medicine, Thessaloniki, Greece. 4. Department of Dermatology, Akdeniz University Faculty of Medicine, Antalya, Turkey. 5. Department of Dermatology, Aarhus University Hospital, Aarhus, Denmark. 6. Department of Dermatology, University Hospital Carl Gustav Carus, Dresden, Germany. 7. Department of Dermatology, Rambam Health Care Campus and The Ruth and Bruce Rappaport, Faculty of Medicine, Haifa, Israel. 8. University of Reims-Champagne-Ardenne, Reims, France. 9. Department of Dermatology, University Hospital of Bern, University of Bern, Bern, Switzerland. 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. Department of Dermatology and Referral Center for Autoimmune Bullous Diseases MALIBUL, Avicenne Hospital, AP-HP, University Paris 13, Bobigny, France. 12. Department of Dermatology, Cristo Re Hospital, Rome, Italy. 13. Autoimmune Bullous Diseases Research Center, Department of Dermatology, Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran. 14. Department of Dermatology, Postgraduate Institute of Medical Education and Research, Chandigarh, India. 15. Autoimmune Blistering Dermatoses Section, Department of Dermatology, Poznan University of Medical Sciences, Poznan, Poland. 16. Department of Dermatology, Medical University, Sofia, Bulgaria. 17. Department of Dermatology, University of Münster, Münster, Germany. 18. Dermatology unit, Azienda ospedaliero -universitaria, Universita di Parma, Parma, Italy. 19. Department of Dermatology, Venereology and Allergology, University Hospital Würzburg, Würzburg, Germany. 20. St John's Institute of Dermatology, London, UK. 21. Department of Dermatology, University Hospital, Technical University Dresden, Dresden, Germany. 22. Department of Dermatology, Allergy and Dermatosurgery, Helios University Hospital Wuppertal, University Witten, Herdecke, Germany. 23. 1st Department of Dermatology-Venereology, Hospital of Skin and Venereal Diseases, Aristotle University Medical School, Thessaloniki, Greece. 24. Department Dermatology and Immunodermatology, Medical University of Warsaw, Warsaw, Poland. 25. National Skin Centre, Singapore, Singapore. 26. Department of Dermatology and Venereology, University Hospital Centre Zagreb, School of Medicine, University of Zagreb, Zagreb, Croatia. 27. Dermatology Unit, Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico, Milan, Italy. 28. Department of Physiopathology and Transplantation, Università degli Studi di Milano, Milan, Italy. 29. Department of Dermatology, Hospital Clínic de Barcelona, Universitat de Barcelona, Barcelona, Spain. 30. Department of Dermatology, Rabin Medical Center, petha-Tikwa and Sackler School of Medicine, Tel Aviv University, Tel aviv, Israel. 31. Department of Dermatology, St George Hospital, University of NSW, Sydney, NSW, Australia. 32. DermoLab, Institute of Dermatology, University of Modena and Reggio Emilia, Modena, Italy. 33. Department of Dermatology, Venereology, and Dermatooncology, Semmelweis University, Budapest, Hungary. 34. Department of Oral Medicine, St John's Institute of Dermatology; Guy's and St Thomas' NHS Foundation Trust, London, UK. 35. Division of Dermatology, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel. 36. Department of Human Molecular Genetics and Biochemistry, Sackler Faculty of Medicine, Tel-Aviv University, Tel Aviv, Israel. 37. Department of Dermatology and Immunodermatology, Medical University of Warsaw, Warsaw, Poland. 38. Department of Dermatology, Karadeniz Technical University Faculty of Medicine, Trabzon, Turkey. 39. Genetics and rare Disease Research Division, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy. 40. Department of Dermatology and Lübeck Institute of Experimental Dermatology (LIED), University of Lübeck, Lübeck, Germany. 41. Department of Dermatology, University of Marburg, Marburg, Germany.
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.
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.
Authors: Hideyuki Ujiie; David Rosmarin; Michael P Schön; Sonja Ständer; Katharina Boch; Martin Metz; Marcus Maurer; Diamant Thaci; Enno Schmidt; Connor Cole; Kyle T Amber; Dario Didona; Michael Hertl; Andreas Recke; Hanna Graßhoff; Alexander Hackel; Anja Schumann; Gabriela Riemekasten; Katja Bieber; Gant Sprow; Joshua Dan; Detlef Zillikens; Tanya Sezin; Angela M Christiano; Kerstin Wolk; Robert Sabat; Khalaf Kridin; Victoria P Werth; Ralf J Ludwig Journal: Front Med (Lausanne) Date: 2022-06-09
Authors: G Genovese; C A Maronese; G Casazza; L Corti; L Venegoni; S Muratori; E Berti; D Fanoni; A V Marzano Journal: Clin Exp Dermatol Date: 2021-08-27 Impact factor: 4.481