Acrodermatitis enteropathica

Only Wilson s disease is associated with copper metabolism. Sickle cell anemia and acrodermatitis enteropathica are associated with Zn metabolism, and geriatric diabetes, with Cr deficiency. 

These features are very similar to those of acrodermatitis enteropathica... 

Evidence for a similar membrane carrier for zinc in humans has been suggested from studies of zinc uptake in acrodermatitis enteropathica. In 1979, using mucosal samples obtained from acrodermatitis patients, Atherton e reported that, at lower lumenal zinc concentrations, brush border zinc uptake was inhibited in these patients, an effect overcome at higher zinc concentrations (48). As a result of these studies, they suggested the possibility of a membrane associated carrier for zinc in the brush border membrane. In the acrodermatitis enteropathica patients, this carrier could be altered or absent. Their hypothesis is supported by findings in rats by both Davies (38) and by this laboratory (43). 

The symptoms of the disease can be ameliorated by dietary zinc supplementation. The differential diagnosis of the disease includes acquired zinc deficiency condition owing to low-zinc level in mother s milk because both conditions are very similar in terms of onset and clinical features. As soon as the low-zinc breastfed infants are weaned, they no longer require supplemental zinc, but acrodermatitis enteropathica patients experience recurrence of symptoms if they discontinue oral zinc therapy. 

Anorexia, dwarfism, anemia, hypogonadism, hyperkeratosis, acrodermatitis, enteropathica, depressed immune response, teratogenic effects. 

A variety of symptoms, not mentioned earlier, have also been associated with zinc deficiency. Zinc deficiency is thought to delay woxmd healing, impair the senses of taste and smell, and impair the functions of the immune system. (The impaired sense of taste is called hypogettsia.) Severe zinc deficiency results in impairment of the immune system and increased infections. These problems have been found in the severe deficiencies that occur in starvation (marasmus) and acrodermatitis enteropathica and in studies with animals. The immune system involves a large array of hormones, hormone receptor proteins, signaling proteins, and DNA-binding proteins, some of which require zinc for activity. It is not clear which of these components is most sensitive to zinc deficiency. 

Grider, A., and Young, E. M. (1996). The acrodermatitis enteropathica mutation transiently affects ziiK metabolism in human fibroblasts. /. Muir. 126, 219-224. 

Clioquinol (5-chloro-7-iodo-8-hydroxyquinoline) was used 30 years ago as an oral antiparasitic agent and to increase the intestinal absorption of zinc in patients with acrodermatitis enteropathica, a genetic disorder of zinc... 

Zinc deficiency occurs in a rare genetic disease, acrodermatitis enteropathica. The disease develops in the early months of life, produces pigmented areas on the elbows, knees, face, and buttocks, and is fatal if left untreated. The disease results from an impairment in zinc absorption. Intestinal disturbances, diarrhea, and growth failure also occur. The disease can be treated successfully by administering oral zinc at two times the RDA (Grider and Yoimg, 1996). 

Failure to include zinc in fluids for total parenteral nutrition (TPN) is another example of iatrogenically induced, conditioned deficiency of zinc. A decline of plasma zinc has been observed in several patients given TPN fluids containing less than 1.25 mg of zinc daily (9,58). The decrease which occurred was probably not attributable to urinary losses. Some patients on TPN have developed a clinical picture resembling acrodermatitis enteropathica. 

Zinc deficiency occurring in patients following penicillamine therapy for Wilsons disease has been reported recently (59). The manifestations consisted of parakeratosis, "dead hair and alopecia, keratitis, and centro-cecal scotoma. The clinical features were similar to those of acrodermatitis enteropathica. Following supplementation with zinc, several clinical manifestations were reversed. 

Moynahan, E. J., Acrodermatitis Enteropathica with Secondary Lactose... 

Following treatment of acrodermatitis enteropathica with diiodohydroxyquinolone (X) the absorption and retention of dietary zinc and other trace metals has been found to be greater than in subjects not receiving the... 

Although genetic defects in the metahoUsm of trace elements are rare, they are nonetheless important because of the information they have provided as to homeostatic control mechanisms. This in turn has led to development of effective therapeutic strategies. The most commonly investigated disorders are those affecting iron (hemochromatosis), copper (Wilson s disease and Menkes syndrome), zinc (acrodermatitis enteropathica), and molybdenum (molybdenum cofactor disease). 

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