Acrodermatitis enteropathica-like eruption during treatment of maple syrup urine disease: report of one case.

We describe a Chinese infant with maple syrup urine disease (MSUD) who had acrodermatitis enteropathica-like skin manifestations, edema, anemia, and diarrhea due to inadequate intake of branched-chain amino acids (BCAAs). A BCAA supplementation resulted in resolution of the eruption. This observation suggested the importance of monitoring plasma amino acids' levels for infants with metabolic disease on special formula.