International Bullous Diseases Group: Consensus on Diagnostic Criteria for Epidermolysis Bullosa Acquisita

C. Prost-Squarcioni; F. Caux; E. Schmidt; M.F. Jonkman; S. Vassileva; S.C. Kim; P. Iranzo; M. Daneshpazhooh; J. Terra; J. Bauer; J. Fairley; R. Hall; M. Hertl; J.S. Lehman; B. Marinovic; A. Patsatsi; D. Zillikens; V. Werth; D.T. Woodley; D.F. Murrell

Disclosures

The British Journal of Dermatology. 2018;179(1):30-41.

Methods

The IBDG met three times during 2015: at the annual meetings of the American Academy of Dermatology in San Francisco (21 experts from 11 countries); the European Society of Dermatological Research in Rotterdam (11 experts from six countries); and the European Academy of Dermatology and Venereology (EADV) in Copenhagen (22 experts from 14 countries). Initial presentations and discussions took place at the first two meetings. After further revisions and discussions at the third meeting, consensus voting took place on the definitions and diagnostic techniques. We defined 'consensus' according to Harmonize Outcome Measures for Eczema by having less than one–third of the key opinion leaders disagreeing on a given diagnostic criterion.^[16,17] Fifty proposals (Table S1; see Supporting Information) were put forward for discussion and voting related to the diagnostic processes involved in EBA. For each proposal, participants could 'agree', be 'undecided' or 'disagree'. Tables summarizing the literature (Tables S2–S6; see Supporting Information) and examples of cases of EBA (Appendix S2; see Supporting Information) were presented for discussion.

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Table 1. Definitions of clinical forms of epidermolysis bullosa acquisita (EBA)
Classical/mechanobullous
One subtype only, characterized by:⁶ trauma–induced lesions (skin fragility)^a bullous/vesicular lesions or erosions encompassed by noninflamed or scarred skin scarring^a and milia formation^a preferably located in trauma–prone sites and the extensor skin surface (dorsal hands,^a elbows,^a knees,^a Achilles tendon, feet^a) possible nail dystrophy^a possible scarring alopecia
Nonclassical/nonmechanobullous
BP–like EBA, defined as an eruption:^{18, 20–24} with features characteristic of BP (pruritus, tense bullae on erythematous or urticarial skin, involvement of trunk and folds) usually mixed with atypical lesions for a BP (skin fragility, bullae on normal skin, milia, involvement of face or extensor area of the limbs)
MM–EBA, defined as a disease that predominantly affects MM lined by squamous epithelium, i.e. MM of:^20–27 mouth pharynx oesophagus epiglottis conjunctiva genitalia anus respiratory tract (in malpighian metaplasia)
Brunsting–Perry–type EBA, defined as a chronic recurrent blistering dermatosis confined to the head and neck^28–32
IgA–EBA, defined as a disease that presents with linear IgA deposits in the BMZ that can be observed by direct IF:^{20, 33, 34} it may resemble LABD it may be more aggressive with mucosal scarring

BP, bullous pemphigoid; MM, mucous membranes; BMZ, basal membrane zone; IF, immunofluorescence; LABD, linear IgA bullous dermatosis. ^aCriteria of Roenigk.

Authors and Disclosures

C. Prost-Squarcioni^1,2,3, F. Caux¹, E. Schmidt⁴, M.F. Jonkman⁵, S. Vassileva⁶, S.C. Kim⁷, P. Iranzo⁸, M. Daneshpazhooh⁹, J. Terra⁵, J. Bauer¹⁰, J. Fairley¹¹, R. Hall¹², M. Hertl¹³, J.S. Lehman¹⁴, B. Marinovic¹⁵, A. Patsatsi¹⁶, D. Zillikens⁴, the International Bullous Diseases Group, V. Werth¹⁷, D.T. Woodley¹⁸ and D.F. Murrell¹⁹

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

Correspondence
Catherine Prost-Squarcioni. E-mail: catherine.prost@aphp.fr

Conflicts of interest
E.S. and D.Z. have received honoraria for lectures from Roche and Fresenius Medical Care, and funding for research and development projects from Euroimmun (E.S., D.Z.), Fresenius Medical Care (D.Z.), Biotest (D.Z.) and Novartis (E.S.). M.H. has received honoraria for participation on advisory boards from Roche, Janssen, Biogen and Novartis, and grants from Biotest, Fresenius and Topas. D.T.W. holds patents with the University of Southern California Stevens Institute on various forms of human recombinant type VII collagen, has received grants for epidermolysis bullosa acquisita and type VII collagen research from the National Institutes of Health, Lotus Tissue Repair and Shire Pharmaceuticals, and has been a consultant for Biofusion, Lotus Tissue Repair and Shire Pharmaceuticals. R.J.F. (co-author) receives fees for consulting from Roche. S.Y. (co-author) received Principal Investigator fees from Roche.