Neonatal acrodermatitis enteropathica-like cutaneous lesions in organic aciduria

Abstract

Cutaneous lesions resembling acrodermatitis enteropathica were described in organic aciduria caused by essential amino acid deficiency. We report two neonatal cases (maple syrup urine disease and methylmalonic acidemia) who developed acrodermatitis enteropathica-like syndrome under protein-free diet. Adequate amino acid supplementation led to prompt improvement of skin lesions.