Pemphigus foliaceus (PF) is the most common disease of the canine pemphigus complex and, according to some authors, the most common autoimmune skin disease in dogs (FMVZ-USP 0,7 cases/month), as is noticed in humans. Genetic factors appear to predispose the development of canine PF and, according to data from several studies, PF is more frequently diagnosed in certain breeds. There is just one report of familial pemphigus foliaceus until now at international literature and the characteristic lesions of this skin disease begun with 6 months of age. This is the first report of this autoimmune disease that lesions appeared in two littermates in adulthood (after two years in both animals). The aim of the present study is to compare the records of the animals according to age, possible triggering factors for PF, type and localization of lesions, clinical signs, presence of pruritus, histopathological features, treatment response and adverse effects with oral glucocorticoids and azathioprine and outcomes. Both animals were attended at Hospital Veterinário (HOVET) in FMVZ/USP. The diagnosis was made by clinical examination, histopathological findings and in one dog, also by indirect immunofluorescence. The animals reported are Akita females, and presented skin lesions after 2 years of age. In that records it wasn’t identified any triggering factor, with both animals presenting the spontaneous form. These animals exhibited erythema, erosions, scaling, and crusting at the time of initial presentation. At that time, the sites of involvement were the dorsal muzzle, planum nasale and pinnae. At the time of diagnosis one animal developed also abdominal pustules. Both animals presented pruritus at the time of diagnosis, none had any clinical sign beside the skin disease but one animal presented concomitant thrombocytopenia. The principal histopathological findings in both cases were subcorneal pustules with neutrophils and acantholytic keratinocytes. One animal was treated with prednisone alone (due to thrombocytopenia) and the another one with prednisone combined with azathioprine. The latter presented ITU and thrombocytopenia as side effects and both animals presented polyuria, polydipsia and weight gain. Both animals were still in therapy, after one year of diagnosis, at the time of the submission of this article. There were made attempts to interrupt the medications without success although reduction in lesion severity and pruritus was seen with immunosuppressive treatment.