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Br J Dermatol. 2018 Jul;179(1):30-41. doi: 10.1111/bjd.16138. Epub 2018 May 8.

International Bullous Diseases Group: consensus on diagnostic criteria for epidermolysis bullosa acquisita.

Author information

1
Department of Dermatology and Referral Center for Autoimmune Bullous Diseases, APHP, Avicenne Hospital, Bobigny, France.
2
Department of Histology, UFR Léonard de Vinci, University Paris 13, Bobigny, France.
3
Department of Pathology, APHP, Avicenne Hospital, Bobigny, France.
4
Department of Dermatology, University of Lübeck, Lübeck, Germany.
5
Department of Dermatology, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands.
6
Department of Dermatology, Medical University of Sofia, Sofia, Bulgaria.
7
Department of Dermatology and Cutaneous Biology Research Institute, Yonsei University College of Medicine, Gangnam Severance Hospital, Seoul, Korea.
8
Department of Dermatology, Hospital Clinic de Barcelona, Facultat de Medicina, Universitat de Barcelona, Barcelona, Spain.
9
Autoimmune Bullous Diseases Research Center, Department of Dermatology, Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran.
10
Division of Molecular Dermatology, Department of Dermatology, Paracelsus Medical University Salzburg, Salzburg, Austria.
11
Department of Dermatology, University of Iowa and Department of Veterans Affairs Medical Center, Iowa City, IA, U.S.A.
12
Department of Dermatology, Duke Medical Center, Durham, NC, U.S.A.
13
Department of Dermatology, University Hospital, Marburg, Germany.
14
Department of Dermatology, Mayo Clinic, Rochester, MN, U.S.A.
15
Department of Dermatology and Venereology, University Hospital Center Zagreb, University of Zagreb School of Medicine, Zagreb, Croatia.
16
Second University Dermatology Department, Aristotle University School of Medicine, Papageorgiou General Hospital, Thessaloniki, Greece.
17
Department of Dermatology, University of Pennsylvania and Philadelphia Department of Veterans Affairs Medical Center, Philadelphia, PA, U.S.A.
18
Department of Dermatology, Keck School of Medicine, University of Southern California, Los Angeles, CA, U.S.A.
19
Department of Dermatology at St George Hospital, University of New South Wales, Sydney, Australia.

Abstract

BACKGROUND:

Epidermolysis bullosa acquisita (EBA) is a complex autoimmune bullous disease disease with variable clinical presentations and multiple possible diagnostic tests, making an international consensus on the diagnosis of EBA essential.

OBJECTIVES:

To obtain an international consensus on the clinical and diagnostic criteria for EBA.

METHODS:

The International Bullous Diseases Group (IBDG) met three times to discuss the clinical and diagnostic criteria for EBA. For the final voting exercise, 22 experts from 14 different countries voted on 50 different items. When > 30% disagreed with a proposal, a discussion was held and re-voting carried out.

RESULTS:

In total, 48 of 50 proposals achieved consensus after discussion. This included nine diagnostic criteria, which are summarized in a flow chart. The IBDG was unable to determine one procedure that would be applicable worldwide. A limitation of the study is that differential diagnosis of bullous systemic lupus erythematosus has not been addressed.

CONCLUSIONS:

This first international consensus conference established generally agreed-upon clinical and laboratory criteria defining the clinical classification of and diagnostic testing for EBA. Holding these voting exercises in person with the possibility of discussion prior to voting has advantages in reaching consensus over Delphi exercises with remote voting.

PMID:
29165796
DOI:
10.1111/bjd.16138

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