Guidelines: Updated Diagnostic Criteria for Epidermolysis Bullosa Acquisita

Epidermolysis bullosa
Epidermolysis bullosa
Members of the International Bullous Disease Group convened to update diagnostic criteria for epidermolysis bullosa acquisita.

The International Bullous Disease Group has developed consensus criteria for the diagnosis of epidermolysis bullosa acquisita (EBA). The guidelines were published in the British Journal of Dermatology.

A total of 38 experts on EBA from 15 countries participated in the consensus. Diagnostic criteria were updated as follows:

  • A bullous disorder within the defined clinical spectrum
  • Histopathology revealing a subepidermal or subepithelial blister
  • A positive direct immunofluorescence microscopy of perilesional skin or mucous membrane with linear immunoglobulin G, C3, immunoglobulin A, and/or immunoglobulin M deposits within the epithelial basement membrane zone
  • Detection of circulating autoantibody against Col7 by immunoblotting, enzyme-linked immunosorbent assay, and/or indirect immunofluorescence microscopy on Col7-expressing human cells
  • Labeling anchoring fibrils by indirect immunoelectron microscopy or negative indirect immunofluorescence microscopy on Col7-deficient skin
  • An u-serration pattern by direct immunofluorescence microscopy
  • Direct immunoelectron microscopy of perilesional skin demonstrating immune deposits within the anchoring fibril zone ± the lower lamina densa
  • In vivo bound immune deposits below type IV collagen by Fluorescent Overlay Antigen Mapping
  • Alternatively for items 4 to 8, dermal labeling by direct and/or indirect immunofluorescence microscopy on salt split skin

Although these criteria are meant to reflect an ideal patient presentation, the tests a particular clinician ultimately selects will most likely be determined by the patient’s clinical presentation, the geography of the practitioner, and which diagnostic test is most logistically accessible.

Overall, the recommendations provide appropriate pathways and a general framework for establishing a diagnosis of EBA, thus allowing the disorder to be distinguished from other, similar, blistering diseases. The guidelines will also be useful for future studies that are designed to define the natural history of and therapeutic outcomes associated with EBA.   

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Prost-Squarcioni C, Caux F, Schmidt E, et al; International Bullous Disease Group. International Bullous Diseases Group – consensus on diagnostic criteria for epidermolysis bullosa acquisita [published online November 22, 2017]. Br J Dermatol. doi:10.1111/bjd.16138