Acrodermatitis Enteropathica Treatment & Prevention Information

By: Juliet Cohen

Acrodermatitis enteropathica is a rare genetic disorder, especially in pediatric and dermatology clinics. It is caused by an inability to absorb sufficient zinc from the diet. Zinc deficiency may be due to inadequate intake, malabsorption, excessive loss, or a combination of these factors. Acquired zinc deficiency can occur with inadequate supply, malabsorption, and low zinc stores. Short bowel syndrome, Crohn's disease, intestinal parasitic infestations, food allergy, eating disorders, and a rare inborn error of metabolism known as non-ketotic hyperglycemia are some of the other causes of acquired zinc deficiency. It is thought that the missing protein may be responsible for decreased zinc uptake and abnormal zinc metabolism. The genetic disorder characterized by intestinal abnormalities that lead to the inability to absorb zinc from the intestine.

There is no cure for the condition. Acrodermatitis enteropathica is occurring in the family context, usually during the first few months after birth. The result is that the disease is rare in breastfed infants. Stomatitis, vesiculobullous lesions on the acral areas, and paronychia may arise later. Other cutaneous findings include palmoplanter psoriasiform, Beau line nail disorder and difficult wound healing with formation of crust-like grained tissue. Hypogonadism, anorexia, predisposition to bacterial and fungial infections, depresssion, mental lethargy and apathy may also occur. Without treatment, the prognosis of acrodermatitis enteropathica is bad, with mortality occurring between four and five years of age.

In the acute phase, irritability and emotional disturbances are evident due to wasting of the brain cortex. It is important to recognize and treat this disorder. The key laboratory finding in acrodermatitis enteropathica is an abnormally low blood zinc level, reflecting impaired zinc uptake. Symptoms usually occur in bottle-fed infants within a few days or weeks after birth and breast-fed infants soon after weaning. Both males and females are equally affected. Red and inflamed patches of dry and scaly skin, particularly around body openings such as the mouth, anus, and eyes, and the skin on elbows, knees, hands, and feet. It may look like atopic dermatitis. The lesions may appear eczematous, or may evolve further into crusted vesicles , bullas or pustules. The skin lesions may be secondarily infected by bacteria such as Staphylococcus aureus or fungi like Candida albicans.

Without treatment, Acrodermatitis enteropathica is fatal and affected individuals may die within a few years. A skin or intestinal mucosal biopsy also may aid the diagnosis. Improvement of the dermatitis with zinc supplementation is another good indicator of deficiency. People with acrodermatitis enteropathica need to be monitored by a healthcare professional to ensure that their level of zinc supplementation is adequate and that the zinc supplements are not inducing a copper deficiency. Individuals with acrodermatitis are usually given over the counter zinc supplements. Oysters, beef, liver, pumpkin seeds, pecans, and Brazil nuts are all high in zinc. However, people with acrodermatitis enteropathica also need to take zinc supplements. Zinc supplements in the amount of 30 to 150 mg per day are used by people with this condition.

Skin Disorders
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