A 46-year-old Brazilian man, with initial pustular lesions, neutrophilic spongiosis and subcorneal cleavage evolved to an atypical pemphigus phenotype, with suprabasal acantholysis. Interestingly, his autoantibody profile, tested by immunofluorescence, immunoblotting, enzyme-linked immunosorbent assay, and immunoprecipitation revealed exclusive IgG anti-desmoglein 1 antibodies in all phases of the disease.