Long-Term Follow-Up of Epidermolysis Bullosa Acquisita with Fluctuations in Serum Cytokine Levels: A Case Report.

We report a case of epidermolysis bullosa acquisita (EBA) in a 47-year-old man who suffered for more than 10 years. To evaluate the longitudinal immunological changes, we measured serum levels of anti-basement membrane zone (BMZ) IgG antibodies by indirect immunofluorescence and anti-type VII collagen antibodies by enzyme-linked immunosorbent assay throughout the clinical course. In addition, serum concentrations of B-cell activating factor (BAFF), interleukin (IL)-9, and IL-10 were measured. Titers of anti-BMZ antibodies and anti-type VII collagen antibodies fluctuated during the follow-up period and corresponded broadly with clinical disease activity. Serum BAFF levels increased before the marked elevation of anti-type VII collagen antibody titers, although they did not closely parallel disease activity. Serum IL-9 showed a transient peak around the time when the anti-type VII collagen antibody titer reached its maximum. Serum IL-10 increased after the peak of the anti-type VII collagen antibody titer. These temporal changes in BAFF, IL-9, and IL-10 observed may provide useful information for understanding the immunological phases of EBA.