ABSTRACT
Acrodermatitis enteropathica (AE) is a rare autosomal recessive disorder caused by zinc deficiency.[1] It can be classified as primary zinc deficiency, genetically based zinc deficiency (classical, AE, acquired zinc deficiency of lactogenic origin), and acquired secondary zinc deficiency.[2] Genetic zinc deficiency is associated with the defects in two zinc transporters, of which one is involved in intestinal zinc uptake ZRT- and IRT-like Protein-4 (ZIP), causing classical and AE. The other is responsible for zinc secretion in breast milk zinc transporter-2 (ZnT) resulting in zinc deficiency of lactogenic origin.[3] Here, we have discussed and reviewed the clinical aspects and probable role of zinc transporters in the manifestation of AE.Acrodermatitis enteropathica (AE), manifests as acral and periorificial dermatitis, alopecia, intractable diarrhoea, and failure to thrive. It is classified as primary zinc deficiency, genetically based deficiency, and acquired secondary deficiency. We hereby report a case of genetically based AE in a one year old child. After reviewing the literature, we have also emphasized the possible role of genetics in the manifestation of AE