Acrodermatitis enteropathica results from a defect in zinc metabolism inherited as an autosomal recessive trait. Zinc is chelated in the gastrointestinal tract by an oligopeptide that is normally destroyed in the bowel. Zinc deficiency results in skin and bowel lesions, as well as alterations in mental status. If the disorder is not treated, death occurs from infection and/or marasmus. Blood zinc levels confirm the diagnosis. Dramatic recovery and normal development occur when dietary zinc is supplemented.