acrodermatitis enteropathica

Last reviewed 01/2018

Acrodermatitis enteropathica (AE) is a disorder of zinc absorption.

  • occurs in two forms: an autosomal recessive inherited form and an acquired form
  • congenital form of AE is a rare genetic disorder characterized by an inborn defect in the gastrointestinal absorption of zinc
    • typically presents when the affected infant is weaned from breast feeding
  • acquired form
    • due to nutritional zinc deficiency, e.g. in premature infants who receive prolonged parenteral alimentation


  • irritability and emotional disturbances are evident in the acute due to atrophy of the brain cortex
  • skin lesions include a periorificial and acral vesiculobullous eruption leading to scaley, sharply demarcated crusted plaques

AE may be fatal without appropriate therapy; the management of this condition is with zinc supplements, either in the diet or added to total parenteral nutrition.

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