Review Article

Clinical Presentation, Pathogenesis, Diagnosis, and Treatment of Epidermolysis Bullosa Acquisita

Table 1

Epidemiology and Ig reactivity in EBA.

Gender distribution 61% female
39% male

Age (median, 75 percentile)47, 30ā€“66 years*

Clinical phenotypesInflammatory phenotypes: 66%
Classical (mechanobullous) phenotype: 33%

Ig reactivity (DIF positive)IgG: 39%
IgA: 19%
IgG and IgA: 35%
Not detected: 7%

Black EBA patients with African descent have a significantly younger age when diagnosed compared to cohorts in Korea or the Netherlands. Abbreviations used: DIF: direct immunofluorescence microscopy. Data is based on 83 EBA patients [6ā€“8].