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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

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.
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