S2k guidelines on diagnosis and treatment of linear IgA dermatosis initiated by the European Academy of Dermatology and Venereology

Author:

Caux Frédéric1ORCID,Patsatsi Aikaterini2ORCID,Karakioulaki Meropi3,Antiga Emiliano4ORCID,Baselga Eulalia5,Borradori Luca6ORCID,Caproni Marzia4ORCID,Cardones Adela R.7,Chandran Nisha Suyien8,Dräger Sören9,Drenovska Kossara10ORCID,Goebeler Matthias11,Günther Claudia12,Hofmann Silke C.13ORCID,Ioannides Dimitrios14ORCID,Joly Pascal15ORCID,Marinović Branka16,Mariotti Elena Biancamaria4ORCID,Marzano Angelo Valerio1718ORCID,Morel Kimberly D.19,Murrell Dedee F.20,Prost Catherine1,Sárdy Miklós2122ORCID,Setterfield Jane2324,Skiljevic Dusan25,Uzun Soner26,Vassileva Snejina10,Zambruno Giovanna27,Schmidt Enno9ORCID

Affiliation:

1. Department of Dermatology and Referral Center for Autoimmune Bullous Diseases, Groupe Hospitalier Paris Seine‐Saint‐Denis AP‐HP and University Paris 13 Bobigny France

2. Autoimmune Bullous Diseases Unit, 2nd Department of Dermatology Aristotle University School of Medicine, Papageorgiou General Hospital Thessaloniki Greece

3. Department of Dermatology and Venereology, Medical Center University Hospital Freiburg Freiburg Germany

4. Department of Health Sciences, Section of Dermatology, Azienda USL Toscana Centro, Rare Diseases Unit, European Reference Network‐Skin Member University of Florence Florence Italy

5. Department of Dermatology Hospital Sant Joan de Deu Barcelona Spain

6. Department of Dermatology University of Bern, Inselspital Berne Switzerland

7. Division of Dermatology, Department of Internal Medicine University of Kansas Medical Center Kansas City Kansas USA

8. Division of Dermatology, Department of Medicine National University Hospital Singapore Singapore

9. Department of Dermatology, Lübeck Institute of Experimental Dermatology (LIED) University of Lübeck Lübeck Germany

10. Department of Dermatology and Venereology University Hospital "Alexandrovska", Medical University‐Sofia Sofia Bulgaria

11. Department of Dermatology, Venereology and Allergology University Hospital Würzburg Würzburg Germany

12. Department of Dermatology University Hospital, Technical University Dresden Dresden Germany

13. Department of Dermatology, Allergy and Dermatosurgery Helios University Hospital Wuppertal, University Witten‐Herdecke Wuppertal Germany

14. 1st Department of Dermatology Aristotle University School of Medicine, Hospital of Skin and Venereal Diseases Thessaloniki Greece

15. Department of Dermatology Rouen University Hospital, INSERM 1234, Normandie University Rouen France

16. Department of Dermatology and Venereology, University Hospital Centre Zagreb, School of Medicine University of Zagreb Zagreb Croatia

17. Dermatology Unit Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico Milan Italy

18. Department of Pathophysiology and Transplantation Università degli Studi di Milano Milan Italy

19. Departments of Dermatology and Pediatrics at Columbia University Medical Center Vagelos College of Physicians and Surgeons New York New York USA

20. Department of Dermatology, St George Hospital University of New South Wales Sydney New South Wales Australia

21. Department of Dermatology, Venereology and Dermatooncology Semmelweis University Budapest Hungary

22. Department of Dermatology and Allergy University Hospital, LMU Munich Germany

23. St John's Institute of Dermatology Guy's and St Thomas' NHS Foundation Trust London UK

24. Centre for Host‐Microbiome Interactions King's College London Faculty of Dentistry, Oral & Craniofacial Sciences London UK

25. Department of Dermatovenereology, Faculty of Medicine University of Belgrade Belgrade Serbia

26. Department of Dermatology Akdeniz University Faculty of Medicine Antalya Turkey

27. Genodermatosis Unit, Translational Pediatrics and Clinical Genetics Research Division Bambino Gesù Children's Hospital, IRCCS Rome Italy

Abstract

AbstractIntroductionLinear IgA dermatosis (LAD) is a rare subepidermal autoimmune bullous disease (AIBD) defined by predominant or exclusive immune deposits of immunoglobulin A at the basement membrane zone of skin or mucous membranes. This disorder is a rare, clinically and immunologically heterogeneous disease occurring both in children and in adults. The aim of this project is to present the main clinical features of LAD, to propose a diagnostic algorithm and provide management guidelines based primarily on experts' opinion because of the lack of large methodologically sound clinical studies.MethodsThese guidelines were initiated by the European Academy of Dermatology and Venereology (EADV) Task Force Autoimmune Bullous Diseases (AIBD). To achieve a broad consensus for these S2k consensus‐based guidelines, a total of 29 experts from different countries, both European and non‐European, including dermatologists, paediatric dermatologists and paediatricians were invited. All members of the guidelines committee agreed to develop consensus‐based (S2k) guidelines. Prior to a first virtual consensus meeting, each of the invited authors elaborated a section of the present guidelines focusing on a selected topic, based on the relevant literature. All drafts were circulated among members of the writing group, and recommendations were discussed and voted during two hybrid consensus meetings.ResultsThe guidelines summarizes evidence‐based and expert opinion‐based recommendations (S2 level) on the diagnosis and treatment of LAD.ConclusionThese guidelines will support dermatologists to improve their knowledge on the diagnosis and management of LAD.

Publisher

Wiley

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