Acrodermatitis Enterohepatica
Acrodermatitis enteropathica (AE)
- It is an autosomal recessive disorder of zinc absorption.
- It presents in infancy, characterized by a triad of acral dermatitis (face, hands, feet, anogenital area), alopecia, and diarrhea.
- These infants have a defect in an intestinal zinc transporter, the human ZIP4 protein encoded on the SLC39A4 gene.
- Mutations in this gene prevents appropriate enteral zinc absorption.
- AE classically presents during infancy on weaning from breast milk to formula or cereal, which have lower zinc bioavailability than breast milk.
- Zn supplementation rapidly improves condition.
- Oral zinc in a dose of about 2 mg/kg/day.
Symptoms of Zinc Deficiency
- Poor Immune system
- Weight loss
- Intercurrent infections
- Hypogonadism in males
- Lack of sexual development in females
- Growth retardation
- Dwarfism
- Delayed puberty in adolescents
- Rough skin
- Poor appetite
- Mental lethargy
- Delayed wound healing
- Short stature
- Diarrhea
Exam Question
- Lifelong treatment required in Acrodermatitis enteropathica.
- Dose of zinc used in acrodermatitis enteropathica is 2 mg/kg.
- Acrodermatitis enteropathica is a rare autosomal recessive disorder caused by an inability to absorb sufficient Zinc from the diet.Symptoms improve with zinc supplementation
- Acrodermatitis enteropathica causes diarrhoea and dermatitis but no dementia.
- Denuded skin around mucocutaneous junction is seen in Acrodermatitis enteropathica .
- A child with alopecia, hyperpigmentation psoriatic dermatitis in genitals & mouth and hypogonadism is likely to be suffering from Zn deficiency.
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