ABSTRACT
While the intake of certain nutrients such as amino acids may be restricted in patients with inborn errors of amino acid metabolism, minimum levels must be maintained in order to allow for protein synthesis during growth. All essential amino acids must be administered and a positive nitrogen balance achieved to allow catabolic events to predominate over anabolic processes. Thus preformed amino acids are supplied and closely monitored to prevent excess or deficits. A rapidly changing tissue like the skin provides early warning signs of essential nutrient deficit. Acrodermatitis-like syndromes have been described in patients suffering from the less common forms of amino acid disorders, in which dietary essential branched chain amino acids are reduced to minimal levels (3). These syndromes are akin to the clinical events triggered by a deficiency of essential
fatty acids (4) or by a zinc deficit (5). Even with the increasing availability of enriched foods intended for patients with a metabolic disorder, infants on semisynthetic diets are particularly prone to nutritional deficiencies. The first sign of scaling dermatitis localized to the face and limbs should always induce suspicion of a deficient diet. Patients with a poor nutritional status may be lethargic and anorectic and fail to gain weight or height. In these stages combined enteral and parenteral support is required to maximize recovery.
