Tehran University of Medical Sciences

Science Communicator Platform

Stay connected! Follow us on X network (Twitter):
Share this content! On (X network) By
Updated S2 K Guidelines for the Management of Bullous Pemphigoid Initiated by the European Academy of Dermatology and Venereology (Eadv) Publisher Pubmed



Borradori L1 ; Van Beek N2 ; Feliciani C3 ; Tedbirt B4 ; Antiga E5 ; Bergman R6, 7 ; Bockle BC8 ; Caproni M9 ; Caux F10 ; Chandran NS11 ; Cianchini G12 ; Daneshpazhooh M13 ; De D14 ; Didona D15 Show All Authors
Authors
  1. Borradori L1
  2. Van Beek N2
  3. Feliciani C3
  4. Tedbirt B4
  5. Antiga E5
  6. Bergman R6, 7
  7. Bockle BC8
  8. Caproni M9
  9. Caux F10
  10. Chandran NS11
  11. Cianchini G12
  12. Daneshpazhooh M13
  13. De D14
  14. Didona D15
  15. Di Zenzo GM16
  16. Dmochowski M17
  17. Drenovska K18
  18. Ehrchen J19
  19. Goebeler M20
  20. Groves R21, 22
  21. Gunther C23
  22. Horvath B24
  23. Hertl M15
  24. Hofmann S25
  25. Ioannides D26
  26. Itzlingermonshi B27, 28
  27. Jedlickova J29, 30
  28. Kowalewski C31
  29. Kridin K32
  30. Lim YL33
  31. Marinovic B34
  32. Marzano AV34, 35
  33. Mascaro JM36
  34. Meijer JM24
  35. Murrell D37
  36. Patsatsi K38
  37. Pincelli C39
  38. Prost C10
  39. Rappersberger K27, 28, 40
  40. Sardy M41, 42
  41. Setterfield J43
  42. Shahid M44
  43. Sprecher E45
  44. Tasanen K46
  45. Uzun S47
  46. Vassileva S44
  47. Vestergaard K48
  48. Vorobyev A2, 49
  49. Vujic I27, 28
  50. Wang G50
  51. Wozniak K32
  52. Yayli S51
  53. Zambruno G52
  54. Zillikens D2, 49
  55. Schmidt E2, 53
  56. Joly P4
Show Affiliations
Authors Affiliations
  1. 1. Department of Dermatology, Inselspital, Bern University Hospital, Bern, Switzerland
  2. 2. Department of Dermatology, University of Lubeck, Lubeck, Germany
  3. 3. Dermatology Unit, Department of Medicine and Surgery, University Hospital, University of Parma, Italy
  4. 4. Department of Dermatology, Rouen University Hospital, Referral Center for Autoimmune Bullous Diseases, Referral Center for Autoimmune Bullous Diseases, Rouen University Hospital, INSERM U1234, Normandie University, Rouen, France
  5. 5. Section of Dermatology, Department of Health Sciences, University of Florence, Florence, Italy
  6. 6. Department of Dermatology, Rambam Health Care Campus, Haifa, Israel
  7. 7. Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa, Israel
  8. 8. Department of Dermatology, Venereology & Allergology, Innsbruck Medical University, Innsbruck, Austria
  9. 9. Department of Health Sciences, Section of Dermatology, AUSL Toscana Centro, Rare Diseases Unit, European Reference Network-Skin Member, University of Florence, Italy
  10. 10. Department of Dermatology and Referral Center for Autoimmune Bullous Diseases, Groupe Hospitalier Paris Seine-Saint-Denis, AP-HP and University Paris 13, Bobigny, France
  11. 11. Department of Medicine, Yong Loo Lin School of Medicine, National University of Singapore, Singapore
  12. 12. Department of Dermatology, Ospedale Classificato Cristo Re, Rome, Italy
  13. 13. Department of Dermatology, Autoimmune Bullous Diseases Research Center, Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran
  14. 14. Department of Dermatology, Venereology and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
  15. 15. Department of Dermatology and Allergology, Philipps University, Marburg, Germany
  16. 16. Laboratory of Molecular and Cell Biology, Istituto Dermopatico dell'Immacolata, IDI-IRCCS, Rome, Italy
  17. 17. Autoimmune Blistering Dermatoses Section, Department of Dermatology, Poznan University of Medical Sciences, Poznan, Poland
  18. 18. Department of Dermatology, Medical University of Sofia, Sofia, Bulgaria
  19. 19. Department of Dermatology, University of Munster, Munster, Germany
  20. 20. Department of Dermatology, Venereology and Allergology, University Hospital Wurzburg, Wurzburg, Germany
  21. 21. St. John's Institute of Dermatology, Viapath Analytics LLP, St. Thomas' Hospital, London, United Kingdom
  22. 22. Division of Genetics and Molecular Medicine, King's College London, Guy's Hospital, London, United Kingdom
  23. 23. Department of Dermatology, Carl Gustav Carus University Hospital, Technische Universitat Dresden, Dresden, Germany
  24. 24. Department of Dermatology, Center for Blistering Diseases, University Medical Center Groningen, University of Groningen, Groningen, Netherlands
  25. 25. Department of Dermatology, Allergy and Dermatosurgery, Helios University Hospital Wuppertal, University Witten, Herdecke, Germany
  26. 26. 1st Department of Dermatology-Venereology, Hospital of Skin and Venereal Diseases, Aristotle University Medical School, Thessaloniki, Greece
  27. 27. Department of Dermatology, Venereology and Allergy, Clinical Center Landstrasse, Academic Teaching Hospital of the Medical University of Vienna, Vienna, Austria
  28. 28. Medical Faculty, The Sigmund Freud Private University, Vienna, Austria
  29. 29. Department of Dermatovenereology, Masaryk University, University Hospital St. Anna, Brno, Czech Republic
  30. 30. Department of Dermatovenereology, University Hospital Brno, Brno, Czech Republic
  31. 31. Department Dermatology and Immunodermatology, Medical University of Warsaw, Warsaw, Poland
  32. 32. National Skin Centre, Singapore, Singapore
  33. 33. Department of Dermatology and Venereology, School of Medicine, University Hospital Centre Zagreb, University of Zagreb, Zagreb, Croatia
  34. 34. Dermatology Unit, Fondazione IRCCS Ca Granda Ospedale Maggiore Policlinico, Milan, Italy
  35. 35. Department of Pathophysiology and Transplantation, Universita degli Studi di Milano, Milan, Italy
  36. 36. Department of Dermatology, Hospital Clinic de Barcelona, Universitat de Barcelona, Barcelona, Spain
  37. 37. Department of Dermatology, St George Hospital, University of New South Wales, Sydney, NSW, Australia
  38. 38. 2nd Department of Dermatology, Autoimmune Bullous Diseases Unit, Aristotle University School of Medicine, Papageorgiou General Hospital, Thessaloniki, Greece
  39. 39. DermoLab, Institute of Dermatology, University of Modena and Reggio Emilia, Modena, Italy
  40. 40. Abteilung Dermatologie, Venerologie und Allergologie, Lehrkrankenhaus der Medizinischen Universitat Wien, Austria
  41. 41. Department of Dermatology and Allergology, Ludwig Maximilian University, Munich, Germany
  42. 42. Department of Dermatology, Venereology and Dermatooncology, Semmelweis University, Budapest, Hungary
  43. 43. Department of Oral Medicine, St John's Institute of Dermatology, Guy's and St Thomas' NHS Foundation Trust, London, United Kingdom
  44. 44. Department of Dermatology, Medical University, Sofia, Bulgaria
  45. 45. Division of Dermatology, Tel Aviv Sourasky Medical Center and Department of Human Molecular Genetics & Biochemistry, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
  46. 46. Department of Dermatology, the PEDEGO Research Unit, University of Oulu and Medical Research Center Oulu, Oulu University Hospital, Oulu, Finland
  47. 47. Department of Dermatology and Venereology, Akdeniz University Faculty of Medicine, Antalya, Turkey
  48. 48. Department of Dermatology, Aarhus University Hospital, Aarhus, Denmark
  49. 49. Center for Research on Inflammation of the Skin, University of Lubeck, Lubeck, Germany
  50. 50. Department of Dermatology, Xijing Hospital, Fourth Military Medical University, Xi'an, China
  51. 51. Department of Dermatology, School of Medicine, Koc University, Istanbul, Turkey
  52. 52. Genetics and Rare Diseases Research Division, Bambino Gesu Children's Hospital, IRCCS, Rome, Italy
  53. 53. Lubeck Institute of Experimental Dermatology (LIED), University of Lubeck, Lubeck, Germany

Source: Journal of the European Academy of Dermatology and Venereology Published:2022


Abstract

Background: Bullous pemphigoid (BP) is the most common autoimmune subepidermal blistering disease of the skin and mucous membranes. This disease typically affects the elderly and presents with itch and localized or, most frequently, generalized bullous lesions. A subset of patients only develops excoriations, prurigo-like lesions, and eczematous and/or urticarial erythematous lesions. The disease, which is significantly associated with neurological disorders, has high morbidity and severely impacts the quality of life. Objectives and methodology: The Autoimmune blistering diseases Task Force of the European Academy of Dermatology and Venereology sought to update the guidelines for the management of BP based on new clinical information, and new evidence on diagnostic tools and interventions. The recommendations are either evidence-based or rely on expert opinion. The degree of consent among all task force members was included. Results: Treatment depends on the severity of BP and patients' comorbidities. High-potency topical corticosteroids are recommended as the mainstay of treatment whenever possible. Oral prednisone at a dose of 0.5 mg/kg/day is a recommended alternative. In case of contraindications or resistance to corticosteroids, immunosuppressive therapies, such as methotrexate, azathioprine, mycophenolate mofetil or mycophenolate acid, may be recommended. The use of doxycycline and dapsone is controversial. They may be recommended, in particular, in patients with contraindications to oral corticosteroids. B-cell-depleting therapy and intravenous immunoglobulins may be considered in treatment-resistant cases. Omalizumab and dupilumab have recently shown promising results. The final version of the guideline was consented to by several patient organizations. Conclusions: The guidelines for the management of BP were updated. They summarize evidence- and expert-based recommendations useful in clinical practice. © 2022 The Authors. Journal of the European Academy of Dermatology and Venereology published by John Wiley & Sons Ltd on behalf of European Academy of Dermatology and Venereology.