[Iatrogenic acrodermatitis enteropathica-like syndrome in leucinosis]

Leucinosis (maple syrup urine disease) is a metabolic disorder caused by an enzymatic deficiency involved in the degradative pathways of the three branched-chain amino acids. We report an observation of acrodermatitis enteropathica-like syndrome induced by essential amino acid deficiency in a child with leucinosis.

A child with leucinosis was referred to our hospital for exfoliative dermatitis of the perioral and anogenital regions associated with diarrhea and pancytopenia. The diagnosis of iatrogenic acrodermatitis enteropathica-like syndrome was confirmed after screening showing isoleucine deficiency. Rapid response was observed after adequate isoleucine supplementation.

The acrodermatitis enteropathica-like eruption in our patient was due to an iatrogenic amino acid nutritional imbalance. Our observation underlines the risk of using a branched-chain amino acid-free formula without adequate supplementation of deficient amino acids. In addition, dietary insufficiency of isoleucine, associated with the treatment of organic aciduria should be added to the causes of acrodermatitis enteropathica-like syndrome.