Fig. 1.

Reported EBA cases and number of publications reporting EBA patients from 1971 to 2016. PubMed was searched using the term “(epidermolysis bullosa acquisita) AND (“1971”[Date - Publication]: “2016”[Date - Publication])”. EBA patients fulfilling the current diagnostic criteria were selected from the retrieved records. A total of 1159 EBA cases (data sets) were identified. Over the years, the number of reported cases ranged from 2 to 5 per year with the exception of 1996–99 and 2011–12, when 11–62 patients were reported per year. The graph displays the cumulative number of EBA patients reported between 1971 and 2016. The number of publications on EBA patients remained relatively constant during the time frame assessed; during this time, 2–6 manuscripts per year were typically published. If publications with a focus on immunological studies, i.e. ELISA development or HLA-associations, are excluded from this analysis, a total of 519 data sets in 194 publications remain. The green arrow indicates the time point when IG deposits were first noticed in EBA patients [3], while the red arrow corresponds to the description of the first [47] and the first commercialized [72] ELISA system detecting autoantibodies directed against COL7