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Acrodermatitis Enteropathica



Acrodermatitis Enteropathica Definition


Acrodermatitis enteropathica is an autosomal recessive disease which affects zinc metabolism. This is characterized by periorificial (in the region of natural orifices) and in the limbs (acral dermatitis), loss of hair (also known as alopecia), and diarrhea. Having acquired zinc deficiency is somewhat similar to acrodermatitis enteropathica because it, too, can be congenital. The other known terms for acrodermatitis enteropathica are: Brandt syndrome, congenital zinc deficiency, and Danbolt-cross syndrome.


Acrodermatitis Enteropathica Frequency


The frequency of the illness in the United States remains unrecorded and unknown but in Denmark, the estimate is at 1 in every 500,000 individuals are affected.


Acrodermatitis Enteropathica Genetics


The mutation on the SLC39A4 gene on chromosome 8q locus 24.3 manifests as the primary cause. This gene appears to predetermine a transmembrane protein which serves as the zinc uptake protein. The characteristics of this disease often start manifesting when an infant is being weaned from mainly thriving on breast milk. The differentiation of acrodermatitis enteropathica from acquired zinc deficit can be hard because each of these conditions present the same symptoms or signs. Some studies have proven that when the mother’s breast milk is low in zinc, the infant that she breastfeeds could manifest zinc deficiency. These studies openly dispute the claim that breast milk can protect the infant from deficiencies in zinc.


Acrodermatitis Enteropathica Mortality/Morbidity


Acrodermatitis enteropathica can be fatal when the disease remains untreated especially within the first few years of the patient’s existence. There was a study made from before that an untreated adult patient has survived the illness. This illness afflicts both the male and female species and no racial partiality exists.


Acrodermatitis Enteropathica Symptoms and Signs


The most common symptoms of acrodermatitis enteropathica start to show up in the first few months after being weaned from breast milk. The following symptoms would soon occur after weaning: erythematous plaques and patches of dry skin (that could also appear scaly); eczematous lesions (which could progress into crusted bullas, vesicles or pustules); anal and oral lesions; hand, scalp and feet lesions; suppurative inflammation could also occur on the nail fold which surrounds the nail plate (also called as paronychia); apparent hair loss on the eyebrows, scalp and eyelashes (or alopecia). The skin lesions could be infected secondarily by staphylococcus aureus which is a bacteria or Candida albicans which is a type of fungus. It is often that these lesions come with acute diarrhea.


Acrodermatitis Enteropathica Treatment


If the disease remains untreated, it could be deadly and patients could die in just a few years. The illness still has no cure. Treatment often includes a dietary zinc supplement which can be taken throughout the entire life of the patient. The zinc supplementation range should be greater than 1-2 milligram per kilogram of bodyweight daily.


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