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. 

In This Article

Abstract and Introduction

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.

Introduction

The International Bullous Diseases Group (IBDG) was formed in 2005 following a research meeting at the National Institutes of Health for experts in blistering diseases to work towards a consensus for the development and validation of definitions and outcome measures in autoimmune bullous diseases (AIBDs).[1] The IBDG focused its efforts initially on pemphigus, with consensus definitions, development and validation of the Pemphigus Disease Area Index and the Autoimmune Bullous Skin Intensity Score.[1,2] Subsequently, the IBDG published consensus definitions for bullous pemphigoid (BP) and proposed the BP Disease Area Index severity tool,[3] and then consensus definitions for mucous membrane (MM) pemphigoid (MMP) and the MMP Disease Area Index.[4] The current project relates to international consensus definitions on diagnostic criteria for epidermolysis bullosa acquisita (EBA).

Two cases of an adult–onset, acquired blistering disease that was reminiscent of patients with hereditary dystrophic epidermolysis bullosa (EB) were reported in 1895 by Elliott.[5] A landmark paper on the subject was published in 1971 by Roenigk et al., who described three new cases of EBA, reviewed the world literature and proposed the first diagnostic criteria for EBA.[6] These criteria were soon modified by the advent of immunofluorescence (IF) and the finding that all patients with EBA had IgG and sometimes C3 deposits in their dermoepidermal junction (DEJ) and that by immunoelectron microscopy (IEM), these DEJ immune deposits were clearly in a different location than immune deposits observed in BP.[7–10] The IgG autoantibodies (autoAbs) accounting for these DEJ immune deposits were found to be autoAbs directed against a 290–kDa protein called type VII collagen (Col7), the major component of anchoring fibrils (AFs) in the DEJ.[11,12] Since these initial observations, the diagnostic testing for EBA has undergone significant refinement.[13–15]

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