Pemphigus herpetiformis (PH) is a rare and unique clinical form of pemphigus foliaceus and pemphigus vulgaris. Patients show autoantibodies against desmoglein 1 and less frequently against desmoglein 3 and desmocollins. We report a 24-year-old woman with a 3-year history of recurrent intensely pruritic erythematous papules and annular plaques localized on the trunk and extremities. In recent months she developed small vesicles around the annular lesions. The histological features showed eosinophilic spongiosis, and direct immunofluorescence demonstrated typical staining of the epidermal intercellular spaces characteristic for pemphigus. There was no mucosal involvement, and hence a diagnosis of PH was established. This patient was unresponsive to dapsone and methotrexate, but she finally experienced remission with prednisone and mycophenolate mofetil.