Introduction Acrodermatitis enteropathica has been recognized as a rare clinical entity distinct from epidermolysis bullosa dystrophica since 1942, when Danbolt and Closs1 classically described the disease. Prior to that time Wende2 and Brandt3 had reported bizarre bullous dermatoses, which in retrospect were acrodermatitis enteropathica. Diiodohydroxyquin (Diadoquin) was found by Schlomovitz4 to be an effective therapeutic agent. Subsequent reports by Dillaha and Lorincz,5 Bloom and Sobel,6 and HodgsonJones7 confirm its successful action. The following case report of acrodermatitis enteropathica is unique. The patient is a 46-year-old woman who has had a mild intermittent form of the disease most of her life. The histopathology of the disorder is reported in detail. Diiodohydroxyquin has resulted in a remission of the disease. Report of a Case A married woman, age 46, was first seen in August, 1956, with a dermatitis involving the distal