Abstract

It is an autoimmune subepidermal bullous disease of infrequent presentation, caused by the presence of autoantibodies that are directed against epidermal basement membrane components, known as BPAG1 Y BPAG2. It occurs mainly in older adults associated with high morbidity and mortality. Clinically it is charac­terized by the appearance of tense blisters on erythematous, edematous and pruritic plaques. For the diag­nosis it is important the correlation of the clinic, histopathological evaluation and the study by immunofluo­rescence. In case of atypical presentations, the diagnosis can become a medical challenge. The treatment is based on the use of immunosuppressants, mainly steroids.