Section 13: Nutritional, Metabolic, and Endocrine Diseases

Acrodermatitis enteropathica This syndrome results from inadequate absorption or dietary intake of zinc. Figure 13-1 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 Fig. 13-2 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.

Acrodermatitis enteropathica Figure 13-3 shows a highly characteristic picture of the cutaneous changes of acrodermatitis enteropathica around the anus, the buttocks, and on the perineum. Note the psoriasiform appearance of this lesion and of those on the feet in Fig. 13-4. 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 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 below normal. Treatment with dietary zinc supplementation leads to a dramatic resolution of all symptoms and, in some cases, must be maintained indefinitely.

Acrodermatitis enteropathica 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 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 then spread. ...