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
Features:
- 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.