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

  • L. Borradori
    Department of Dermatology Inselspital, Bern University Hospital Bern Switzerland
  • N. Van Beek
    Department of Dermatology University of Lübeck Lübeck Germany
  • C. Feliciani
    Dermatology Unit Department of Medicine and Surgery University Hospital University of Parma Italy
  • B. Tedbirt
    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
  • E. Antiga
    Section of Dermatology Department of Health Sciences University of Florence Florence Italy
  • R. Bergman
    Department of Dermatology Rambam Health Care Campus Haifa Israel
  • B. C. Böckle
    Department of Dermatology, Venereology & Allergology Innsbruck Medical University Innsbruck Austria
  • M. Caproni
    Department of Health Sciences Section of Dermatology AUSL Toscana Centro, Rare Diseases Unit, European Reference Network‐Skin Member University of Florence Italy
  • F. Caux
    Department of Dermatology and Referral Center for Autoimmune Bullous Diseases Groupe Hospitalier Paris Seine‐Saint‐Denis AP‐HP and University Paris 13 Bobigny France
  • N.S. Chandran
    Department of Medicine Yong Loo Lin School of Medicine National University of Singapore Singapore
  • G. Cianchini
    Department of Dermatology Ospedale Classificato Cristo Re Rome Italy
  • M. Daneshpazhooh
    Department of Dermatology, Autoimmune Bullous Diseases Research Center Razi Hospital, Tehran University of Medical Sciences Tehran Iran
  • D. De
    Department of Dermatology, Venereology and Leprology Postgraduate Institute of Medical Education and Research Chandigarh India
  • D. Didona
    Department of Dermatology and Allergology Philipps University Marburg Germany
  • G. M. Di Zenzo
    Laboratory of Molecular and Cell Biology Istituto Dermopatico dell'Immacolata IDI‐IRCCS Rome Italy
  • M. Dmochowski
    Autoimmune Blistering Dermatoses Section Department of Dermatology Poznan University of Medical Sciences Poznan Poland
  • K. Drenovska
    Department of Dermatology Medical University of Sofia Sofia Bulgaria
  • J. Ehrchen
    Department of Dermatology University of Münster Münster Germany
  • M. Goebeler
    Department of Dermatology, Venereology and Allergology University Hospital Würzburg Würzburg Germany
  • R. Groves
    St. John's Institute of Dermatology, Viapath Analytics LLP St. Thomas' Hospital London UK
  • C. Günther
    Department of Dermatology Carl Gustav Carus University Hospital Technische Universität Dresden Dresden Germany
  • B. Horvath
    Department of Dermatology Center for Blistering Diseases University Medical Center Groningen, University of Groningen Groningen the Netherlands
  • M. Hertl
    Department of Dermatology and Allergology Philipps University Marburg Germany
  • S. Hofmann
    Department of Dermatology, Allergy and Dermatosurgery Helios University Hospital Wuppertal, University Witten Herdecke Germany
  • D. Ioannides
    1<sup>st</sup> Department of Dermatology‐Venereology, Hospital of Skin and Venereal Diseases Aristotle University Medical School Thessaloniki Greece
  • B. Itzlinger‐Monshi
    Department of Dermatology, Venereology and Allergy Clinical Center Landstrasse Academic Teaching Hospital of the Medical University of Vienna Vienna Austria
  • J. Jedličková
    Department of Dermatovenereology Masaryk University, University Hospital St. Anna Brno
  • C. Kowalewski
    Department Dermatology and Immunodermatology Medical University of Warsaw Warsaw Poland
  • K. Kridin
    National Skin Centre Singapore Singapore
  • Y. L. Lim
    Department of Dermatology and Venereology School of Medicine University Hospital Centre Zagreb, University of Zagreb Zagreb Croatia
  • B. Marinovic
    Dermatology Unit Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico Milan Italy
  • A. V. Marzano
    Dermatology Unit Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico Milan Italy
  • J.‐M. Mascaro
    Department of Dermatology Hospital Clínic de Barcelona, Universitat de Barcelona Barcelona Spain
  • J.M. Meijer
    Department of Dermatology Center for Blistering Diseases University Medical Center Groningen, University of Groningen Groningen the Netherlands
  • D. Murrell
    Department of Dermatology St George Hospital University of New South Wales Sydney New South Wales Australia
  • K. Patsatsi
    2<sup>nd</sup> Department of Dermatology Autoimmune Bullous Diseases Unit Aristotle University School of Medicine Papageorgiou General Hospital Thessaloniki Greece
  • C. Pincelli
    DermoLab Institute of Dermatology University of Modena and Reggio Emilia Modena Italy
  • C. Prost
    Department of Dermatology and Referral Center for Autoimmune Bullous Diseases Groupe Hospitalier Paris Seine‐Saint‐Denis AP‐HP and University Paris 13 Bobigny France
  • K. Rappersberger
    Department of Dermatology, Venereology and Allergy Clinical Center Landstrasse Academic Teaching Hospital of the Medical University of Vienna Vienna Austria
  • M. Sárdy
    Department of Dermatology and Allergology Ludwig Maximilian University Munich Germany
  • J. Setterfield
    Department of Oral Medicine St John's Institute of Dermatology Guy's and St Thomas' NHS Foundation Trust London UK
  • M. Shahid
    Department of Dermatology Medical University Sofia Bulgaria
  • E. Sprecher
    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
  • K. Tasanen
    Department of Dermatology the PEDEGO Research Unit University of Oulu and Medical Research Center Oulu Oulu University Hospital Oulu Finland
  • S. Uzun
    Department of Dermatology and Venereology Akdeniz University Faculty of Medicine Antalya Turkey
  • S. Vassileva
    Department of Dermatology Medical University Sofia Bulgaria
  • K. Vestergaard
    Department of Dermatology Aarhus University Hospital Aarhus Denmark
  • A. Vorobyev
    Department of Dermatology University of Lübeck Lübeck Germany
  • I. Vujic
    Department of Dermatology, Venereology and Allergy Clinical Center Landstrasse Academic Teaching Hospital of the Medical University of Vienna Vienna Austria
  • G. Wang
    Department of Dermatology Xijing Hospital, Fourth Military Medical University Xi'an China
  • K. Wozniak
    National Skin Centre Singapore Singapore
  • S. Yayli
    Department of Dermatology, School of Medicine Koç University Istanbul Turkey
  • G. Zambruno
    Genetics and Rare Diseases Research Division Bambino Gesù Children's Hospital, IRCCS Rome Italy
  • D. Zillikens
    Department of Dermatology University of Lübeck Lübeck Germany
  • E. Schmidt
    Department of Dermatology University of Lübeck Lübeck Germany
  • P. Joly
    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

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<jats:title>Abstract</jats:title><jats:sec><jats:title>Background</jats:title><jats:p>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.</jats:p></jats:sec><jats:sec><jats:title>Objectives and methodology</jats:title><jats:p>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.</jats:p></jats:sec><jats:sec><jats:title>Results</jats:title><jats:p>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.</jats:p></jats:sec><jats:sec><jats:title>Conclusions</jats:title><jats:p>The guidelines for the management of BP were updated. They summarize evidence‐ and expert‐based recommendations useful in clinical practice.</jats:p></jats:sec>

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