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. 2022 Oct;36(10):1689-1704.
doi: 10.1111/jdv.18220. Epub 2022 Jun 29.

Updated S2 K guidelines for the management of bullous pemphigoid initiated by the European Academy of Dermatology and Venereology (EADV)

L Borradori  1 , N Van Beek  2 , C Feliciani  3 , B Tedbirt  4 , E Antiga  5 , R Bergman  6   7 , B C Böckle  8 , M Caproni  9 , F Caux  10 , N S Chandran  11 , G Cianchini  12 , M Daneshpazhooh  13 , D De  14 , D Didona  15 , G M Di Zenzo  16 , M Dmochowski  17 , K Drenovska  18 , J Ehrchen  19 , M Goebeler  20 , R Groves  21   22 , C Günther  23 , B Horvath  24 , M Hertl  15 , S Hofmann  25 , D Ioannides  26 , B Itzlinger-Monshi  27   28 , J Jedličková  29   30 , C Kowalewski  31 , K Kridin  32 , Y L Lim  33 , B Marinovic  34 , A V Marzano  34   35 , J-M Mascaro  36 , J M Meijer  24 , D Murrell  37 , K Patsatsi  38 , C Pincelli  39 , C Prost  10 , K Rappersberger  27   28   40 , M Sárdy  41   42 , J Setterfield  43 , M Shahid  44 , E Sprecher  45 , K Tasanen  46 , S Uzun  47 , S Vassileva  44 , K Vestergaard  48 , A Vorobyev  2   49 , I Vujic  27   28 , G Wang  50 , K Wozniak  32 , S Yayli  51 , G Zambruno  52 , D Zillikens  2   49 , E Schmidt  2   53 , P Joly  4
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Free article

Updated S2 K guidelines for the management of bullous pemphigoid initiated by the European Academy of Dermatology and Venereology (EADV)

L Borradori et al. J Eur Acad Dermatol Venereol. 2022 Oct.
Free article

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.

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