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Hereditary acrodermatitis enteropathica - Anogenital in
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Other Resources UpToDate PubMed

Hereditary acrodermatitis enteropathica - Anogenital in

See also in: Overview
Contributors: Mary Spencer MD, Ann Lenane MD, Sireesha Reddy MD, Amy Swerdlin MD, Manasi Kadam Ladrigan MD, Carol Berkowitz MD
Other Resources UpToDate PubMed

Synopsis

Hereditary acrodermatitis enteropathica is an eczematous dermatitis on the face, hands, feet, and anogenital area, caused by zinc deficiency. It is attributed to an autosomal recessive inherited disorder of zinc absorption, due to abnormal zinc binding ligands. It usually occurs days to weeks after birth in infants given cow's milk. In breast-fed infants, it can occur soon after the transition to cow's milk.

Identical clinical findings may result from acquired zinc deficiency due to other causes. It may be seen in premature infants who are exclusively breast fed in the setting of increased metabolic demands and declining levels of zinc in the mother's breast milk. Zinc deficiency has rarely been linked to a defect in the transfer of zinc into breast milk. Historically, it was seen in infants receiving parenteral nutrition, although zinc supplementation has now become routine. Since late 2012, however, injectable zinc, which is used in parenteral nutrition formulations, periodically has been on the drug shortage list in the United States; thus clinicians should be aware of the possibility that hereditary acrodermatitis enteropathica can be mimicked in infants receiving parenteral nutrition formulations because of a disease such as biliary atresia.

Codes

ICD10CM:
E83.2 – Disorders of zinc metabolism

SNOMEDCT:
37702000 – Hereditary acrodermatitis enteropathica

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Last Updated:09/09/2020
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Hereditary acrodermatitis enteropathica - Anogenital in
See also in: Overview
A medical illustration showing key findings of Hereditary acrodermatitis enteropathica : Diarrhea, Rash, Acral distribution, Failure to thrive, Irritability, Skin erosion
Clinical image of Hereditary acrodermatitis enteropathica - imageId=524030. Click to open in gallery.  caption: 'Patchy erythema on the cheek and ear.'
Patchy erythema on the cheek and ear.
Copyright © 2024 VisualDx®. All rights reserved.