Hereditary acrodermatitis enteropathica - Anogenital in
See also in: OverviewAlerts and Notices
Important News & Links
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.
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
E83.2 – Disorders of zinc metabolism
SNOMEDCT:
37702000 – Hereditary acrodermatitis enteropathica
Look For
Subscription Required
Diagnostic Pearls
Subscription Required
Differential Diagnosis & Pitfalls
To perform a comparison, select diagnoses from the classic differential
Subscription Required
Best Tests
Subscription Required
Management Pearls
Subscription Required
Therapy
Subscription Required
References
Subscription Required
Last Updated:09/09/2020
Hereditary acrodermatitis enteropathica - Anogenital in
See also in: Overview