Epidermolysis bullosa acquisita: diagnostic difficulties

Introduction:
Epidermolysis bullosa acquisita is a blistering disease in which the autoimmune response is directed against collagen VII epitopes.

Case report:
A 50-year-old patient was admitted to the Department due to erythematous erosions and single blisters. The direct immunofluorescence study from a perilesional biopsy showed in vivo bound linear IgG deposits and focal C3 along the dermal-epidermal junction; however, the characteristic u-serrated pattern was not observed. No circulating antibodies were found in the serum. The direct immunofluorescence study performed on salt-split skin revealed immune deposits located along the floor and focally within the roof of the artificial blister. To determine the final diagnosis, type VII collagen was mapped on patient’s salt-split skin using double-labelling with anti-collagen VII antibodies and serum from a seropositive epidermolysis bullosa acquisita patient. The overlapping of the identical images was obtained.

Conclusions:
Marking the distribution of collagen VII on salt-split skin enabled the diagnosis.