Acrodermatitis enteropathica-like cutaneous lesions have been reported in patients with some metabolic disorders that are treated with a low-protein diet. These patients usually have low blood levels of some amino acids, especially isoleucine. We describe two patients who evolved with eruptions resembling acrodermatitis enteropathica while undergoing treatment for maple syrup urine disease, without zinc deficiency. Isoleucine supplementation led to a prompt improvement of the skin disorder.
Acrodermatitis; Isoleucine; Maple syrup urine disease
