Acrodermatitis enteropathica (AE) is a genetic disorder of zinc absorption, presenting in infancy, characterized by a triad of acral dermatitis (face, hands, feet, anogenital area), alopecia, and diarrhea; nearly identical clinical findings occur in other individuals with acquired zinc deficiency (AZD) due either to dietary deficiency or failure of intestinal absorption.
Causes of Zinc Deficiency and Acrodermatitis Enteropathica
In AE, patients do not absorb enough zinc from the diet. The specific ligand involved in basic transport mechanisms for zinc that might be abnormal in AE is not known. The defect appears to be somewhere in the early stages of zinc nutriture, where zinc is presented to the intestinal brush border. This defect can be overcome by increased zinc supply in the diet. It is not known how zinc deficiency leads to skin and other lesions.
Symptoms of Zinc Deficiency and Acrodermatitis Enteropathica
- Patches evolve into crusted, blistered, pus-filled and eroded lesions.
- There is usually a sharp demarcation between the affected area and normal skin.
- Skin around the fingernails may be red and swollen.
- Impaired wound healing.
- Skin around nails becomes inflamed (paronychia) and there may be nail ridging.
- Diffuse hair loss on the scalp, eyebrows and, eyelashes.
- Sensitivity to light
- Loss of appetite
- Depressed mood
- Growth failure
Diagnosis
Clinical diagnosis confirmed by zinc blood levels and histopathology.
Treatment
Dietary or IV supplementation with zinc salts in 2 to 3 times the required daily amount restores normal zinc status in days to weeks.
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