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Acquired acrodermatitis enteropathica in Child
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Acquired acrodermatitis enteropathica in Child

Contributors: David O'Connell MD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Acquired acrodermatitis enteropathica (AAE) is an acquired condition that occurs as a result of zinc deficiency. In AAE, skin manifestations may be accompanied by alopecia and diarrhea, but this classic triad is present in only 20% of cases. In mild to moderate deficiency, the clinical picture is that of thin, scaly plaques that may resemble psoriasis. With a more severe deficiency, it will manifest as a periorificial, acral, and intertriginous dermatitis that has eczematous features, but may also be vesiculobullous or even pustular. The eruption tends to be symmetrical.

It appears that zinc is required for the orderly proliferation of keratinocytes. Additionally, low tissue zinc is associated with the overproduction of inflammatory cytokines and reactive mediators.

The disease occurs in all age groups and there is no gender predilection.

Decreased absorption of zinc is associated with inadequate dietary intake due to low caloric diets, anorexia nervosa, vegetarian diets, and diets high in phytates that bind zinc (typical of cereal grain-based diets common to parts of the Middle East and North Africa). Total parenteral nutrition (TPN) with inadequate zinc supplement is a known cause of deficiency. Malabsorption related to inflammatory bowel disease, celiac sprue, cystic fibrosis, and numerous bariatric procedures also leads to zinc deficiency. Chronic diarrhea, along with chronic liver and/or renal disease (nephrotic syndrome), are each risk factors for deficiency.

Medications associated with zinc deficiency include proton pump inhibitors (decrease absorption) and loop diuretics (increase loss). Some angiotensin converting enzyme (ACE) inhibitors and calcium channel blockers have also been implicated.

Identical clinical findings may result from hereditary acrodermatitis enteropathica, a rare autosomal recessive disease caused by abnormal zinc absorption due to a transport deficiency across the small intestine.

Codes

ICD10CM:
E60 – Dietary zinc deficiency

SNOMEDCT:
12602008 – Acquired acrodermatitis enteropathica

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Last Reviewed:03/03/2021
Last Updated:03/15/2021
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Acquired acrodermatitis enteropathica in Child
Acquired acrodermatitis enteropathica : Diarrhea, Acral distribution, Crust, Intertriginous distribution, Perioral, Scaly plaque, Skin erosion, Toes
Clinical image of Acquired acrodermatitis enteropathica
Fairly well-demarcated eroded, crusted, and scaly plaques on the lower legs.
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