Brazilian pemphigus foliaceus is a blistering skin disease endemic to central and southern areas of South America. In this study of skin biopsy specimens from 14 patients we present evidence that complement and immunoglobulins were present by direct immunofluorescence in the epidermal intercellular spaces in all patients. Eight of 14 patients had granular deposits of C3 in the basement membrane zone. By indirect immunofluorescence, serum samples from all 19 patients tested demonstrated the presence of circulating IgG autoantibody. Autoantibodies deposited in the intercellular spaces in titers ranging from 1:10 to more than 1:1280, and the titers drastically decreased during treatment. This is the first study to demonstrate complement deposition in the skin in Brazilian pemphigus foliaceus.