Acrodermatitis enteropathica (AE) is a familial syndrome with a high incidence of morbidity and mortality. The pathogenesis is unknown. Diiodohydroxyquin and related preparations have been the mainstay of treatment for the past 20 years. Recent evidence indicates that zinc therapy given orally may be the most effective treatment. We will describe the case of a 21-year-old woman who had had AE since she was 3 months old. Successful results were obtained with zinc therapy alone.

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