Acrodermatitis enterohepatica

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]


Overview

Acrodermatitis enteropathica occurs in two forms: an autosomal recessive inherited form and an acquired form. The congenital form of AE is a rare genetic disorder characterized by an inborn defect in the gastrointestinal absorption of zinc. Manifestations of the disease typically present when the affected infant is weaned from breast feeding. The acquired form is due to nutritional zinc deficiency, e.g. in premature infants who receive prolonged parenteral alimentation. The characteristic skin lesions include a periorificial and acral vesiculobullous eruption leading to scaley, sharply demarcated crusted plaques. In the acute phase, irritability and emotional disturbances are evident due to wasting (atrophy) of the brain cortex.

Other names include Acrodermatitis enteropathica, Acrodermatitis enteropathica syndrome, Brandt syndrome, Brandt's syndrome, Danbolt - Close syndrome, Danbolt syndrome, Danbolt-Closs syndrome, Danbolt's syndrome, Hereditary acrodermatitis enterohepatica, Primary zinc malabsorption, Primary zinc malabsorption syndrome, Syndrome, Brandt's, Syndrome, Danbolt, Syndrome, Danbolt-Closs


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