Acrodermatitis acidemica secondary to malnutrition in glutaric aciduria type I

We encountered a patient with glutaric aciduria type I (GA-I) associated wi th skin lesions resembling acrodermatitis enteropathica (AE). This child wa s being fed with a low-protein diet when the skin disorder developed. A def iciency in plasma levels of essential amino acids, particularly isoleucine, and zinc was confirmed. Supplementation of a high-caloric, protein-rich di et together with zinc, selenium and vitamins led to a prompt improvement of the skin lesions. We assume that in our patient the skin lesions were the result of malnutrition, rather than being primarily associated with the und erlying metabolic disease. To our knowledge, no other report is so far avai lable concerning GA-I complicated by skin eruptions.