INFLAMMATORY EPIDERMOLYSIS-BULLOSA ACQUIS ITA

Epidermolysis bullosa acquisita (EBA) is an autoimmune subepidermal bl istering disease, which can present in various clinical forms. We repo rt on a 73-year-old male patient with the inflammatory type of EBA, wh ich is clinically similar to bullous pemphigoid. Histological examinat ion revealed subepidermal blister formation, and direct immunofluoresc ence of perilesional skin revealed a linear deposition of IgG and C3 a t the basement membrane. Indirect immunofluorescence with 0.9% NaCl-se parated human skin as substrate showed the presence of circulating ant ibodies binding to the base of the artificial blister at a titre of 1: 320. Indirect immunoelectronmicroscopy revealed immune deposits locali zed in the area of the sublamina densa. Immunoblotting of dermal extra cts disclosed binding of serum antibodies to a 290-kDa protein. System ic therapy with diaminodiphenyl sulphone, initially in combination wit h corticosteroids, resulted in complete healing of the skin lesions. W ith reference to this case report, we discuss the clinical pictures po ssible in EBA, the differential diagnosis an the treatment options.