by andywalsh | May 16, 2019 11:18 pm
It is defined as a condition in which there is a metabolic disorder which affects the uptake of zinc. It is autosomal recessive in nature. It shows peri orifical and acral dermatitis which is present near the natural orifices and around the limbs. It also shows the loss of hair along with the diarrhea. The symptoms are similar to which are present in the zinc deficiency syndrome. It is also related to the congenital zinc deficiency. It is also referred as a Brandt syndrome and Danbolt cross syndrome.
There is a mutation in the SLC39A4 gene which is present on the chromosome 8 q 24. It is responsible for this disorder. This gene encodes a trans membrane protein which serve as a zinc uptake protein. This disease starts early in the infant and when he is weaned from the breast milk. It has led to the belief that the human milk contains a substance which helps in the uptake of zinc. It prevents the disease when the infant is on breast milk.
They appear during the first few months of life. During this time the infant discontinues the milk and is followed by the erythematous patches. There are plaques of dry and scaly skin. The lesion appears as an eczematous and can involve further crusted vesicles, bullas and pustules. It may occur at the mouth or anus. It may also involve the hand, feet and scalp. In the nail fold one can see a suppurative inflammation which is present around the nail plate. It is known as the paronchyia. The alopecia along with the loss of hair from the eyelids, scalps and eye brows is also seen. The skin lesions may also be affected with the bacteria later on which include the staphylococcus aureus or fungi like candida albicans. It is also accompanied by the diarrhea.
If it is not treated it can lead to the death of the individual. It may happen after few years and there is no cure for this disorder. The treatment includes the lifelong dietary zinc supplements around 1 to 2 mg per kg of the body weight.
Source URL: https://alldiseases.org/acrodermatitis-enteropathica/
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