John Libbey Eurotext




Acrodermatitis acidemica secondary to malnutrition in glutaric aciduria type I
European Journal of Dermatology. Volume 11, Number 3, 244-6, May - June 2001, Cas cliniques
Free Article
Author(s) : S. Niiyama, S. Koelker, I. Degen, G.F. Hoffmann, R. Happle, R. Hoffmann
Summary : We encountered a patient with glutaric aciduria type I (GA-I) associated with skin lesions resembling acrodermatitis enteropathica (AE). This child was being fed with a low-protein diet when the skin disorder developed. A deficiency in plasma levels of essential amino acids, particularly isoleucine, and zinc was confirmed. Supplementation of a high-caloric, protein-rich diet 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 underlying metabolic disease. To our knowledge, no other report is so far available concerning GA-I complicated by skin eruptions.
Keywords : acrodermatitis enteropathica, amino acids, metabolism, isoleucine.

Acrodermatitis acidemica secondary to malnutrition in glutaric aciduria type I