Acrodermatitis enteropathica

Abstract

Acrodermatitis enteropathica ADE (AE; MIM# 201100) is a rare autosomal recessive disorder of zinc deficiency. Although a deficiency dermatitis caused by low dietary zinc has the exact clinical and histologic features of ADE. Individuals with ADE suffer from severe zinc deficiency derived from a defective uptake of zinc in the duodenum and jejunum. Characteristic lesions of ADE and acquired deficiency dermatitis caused by low zinc, include dry scaly eczematous plaques of the face, scalp and anogenital area, as well as generalized alopecia and diarrhea. The diagnosis is based on the clinical picture and estimation of plasma zinc concentration. Low levels of serum alkaline phosphatase may support a diagnosis of zinc deficiency. A skin or intestinal mucosal biopsy also may aid the diagnosis. The management of a patient with ADE of acquired zinc deficiency is by zinc supplementation. Response to treatment may take days to weeks, depending on the degree of zinc depletion. The patient should be maintained on lifelong oral zinc supplementation after the diagnosis unless the underlying problem is remediable.