Acrodermatitis enteropathica (AE) is a rare autosomal recessive disorder characterized by periorificial and acral dermatitis, alopecia, growth failure, gastrointestinal disturbance, and diarrhea. Symptoms of AE occur within the first few months after birth and tend to appear in nonbreastfed infants or in infants shortly after discontinuation of breastfeeding. Ocular complications include lid and surface involvement as well as secondary infections. 
The nature of the metabolic defect is currently attributed to zinc absorption. The AE mutation creates reduced zinc uptake and abnormal zinc metabolism in human fibroblasts. The gene defect is located on chromosome 8, characterized as the intestinal zinc transporter gene, or SLC39A4 gene, at locus 8q24.3. [2, 3, 4, 5]
It is estimated that 1 in 500,000 people in Denmark are affected.
AE is lethal, usually within the first few years of life, if left untreated. However, an untreated adult survivor was reported.
No racial predilection exists.
No sexual preference exists.
AE appears in the first few months after birth or after cessation of breastfeeding.
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