Intestinal Acrodermatitis: Origins, Symptoms, Prospects, and Beyond
Acrodermatitis enteropathica is a little-known but important condition that affects the skin and overall health. This rare disorder, primarily caused by inherited mutations in the SLC39A4 gene, can lead to a deficiency in zinc, an essential mineral for numerous bodily functions.
The inherited form of acrodermatitis enteropathica typically presents early in infancy, often after weaning, and if left untreated, can be life-threatening, particularly for infants with primary acrodermatitis enteropathica. Symptoms of the condition include a perioral rash, abnormal nails, hair loss, light sensitivity, loss of appetite, diarrhea, fatty substances present in the feces, slow or reduced growth, irritability or excessive crying in infants, low mood and emotional affect, decreased taste, decreased function of the testes, and potential skin inflammation and rashes.
However, it's important to note that a differential diagnosis is necessary to rule out other conditions that may resemble acrodermatitis enteropathica, such as atopic eczema, celiac sprue, psoriasis, thermal burn, pellagra, superinfection with Candida, and other conditions.
The diagnosis of acrodermatitis enteropathica can be confirmed by testing the zinc levels in the blood, other substances such as alkaline phosphate and albumin, and possibly the zinc levels in the hair and urine. Treatment typically involves zinc supplements, with a high dose for the inherited form and a lower dose for the acquired form.
Acrodermatitis enteropathica can also occur due to acquired zinc deficiency, which is not the classic form of the disorder but shares some symptoms. Risk factors for acquired forms include inadequate dietary zinc intake, conditions causing malabsorption or increased zinc loss, and increased metabolic demand for zinc due to illness or developmental stages.
It's crucial to remember that a zinc overdose is possible, with symptoms including vomiting, diarrhea, stomach cramps, and lightheadedness. As always, if you suspect you or someone else may have acrodermatitis enteropathica, it's essential to seek medical advice promptly.
In summary, the main cause of acrodermatitis enteropathica is a genetic defect impairing zinc absorption, while clinically similar conditions can arise from acquired zinc deficiency due to nutritional, metabolic, or gastrointestinal causes. Early diagnosis and treatment can significantly improve the outlook for individuals with acrodermatitis enteropathica, with most experiencing improvements in symptoms within a few days to a month.
