Section 10. Nutritional and Metabolic Disorders

This syndrome results from inadequate absorption or dietary intake of zinc. Figure 10-1 shows erythema, crusting, and fissuring of the perioral skin and cheeks. The eruption that is pictured here may be preceded by blisters. Other features of acrodermatitis enteropathica include stomatitis, paronychia, and alopecia.

The diaper area lesion that is seen in this figure is diffusely erythematous and has a sharply marginated border on the abdomen. Acrodermatitis enteropathica may be inherited in an autosomal recessive fashion. This form of the disease seems to be related to an inability to absorb zinc.

Figure 10-3 shows a highly characteristic picture of the cutaneous changes of acrodermatitis enteropathica around the anus, the buttocks, and on the perineum. Note how psoriasiform this lesion and those on the feet in Fig. 10-4 are. The full-blown picture of acrodermatitis enteropathica goes far beyond the typical changes of skin and hair. Affected children have severe diarrhea, growth retardation, and irritability. Without treatment, the disease follows a progressive course and may even be fatal. The crucial relationship between acrodermatitis enteropathica and zinc deficiency was discovered in 1973. The child with suspected acrodermatitis enteropathica should be evaluated for a low zinc level or a low alkaline phosphatase level when zinc levels are normal or low normal. (Blood must be drawn in plastic, nonrubber stoppered tubes to avoid the possibility of zinc contamination.) Treatment with dietary zinc supplementation leads to a dramatic resolution of all symptoms and must be maintained indefinitely.

Acquired acrodermatitis enteropathica is seen in infants who have received parenteral alimentation lacking sufficient zinc and, rarely, in breast-fed premature infants who have larger zinc requirements. Occasionally, acrodermatitis enteropathica in a full-term breast-fed infant may be the result of low levels of zinc in the breast milk.

The patient with acquired acrodermatitis enteropathica requires temporary zinc replacement. The differential diagnosis of this eruption includes psoriasis, biotin and multiple carboxylase deficiencies, essential fatty acid deficiencies, and cystic fibrosis.

Kwashiorkor is a type of protein energy malnutrition. It is seen most commonly in developing countries, and onset tends to occur after weaning. At that time, the balance of protein and carbohydrate in breast milk is replaced by a diet that contains almost exclusively carbohydrates. The initial signs are diarrhea, irritability, and edema of the hands and feet. Small dark patches appear at pressure points of the elbows, ankles, wrists, and knees, and ...