Medical Definition of Acrodermatitis enteropathica

  • Medical Author:
    William C. Shiel Jr., MD, FACP, FACR

    Dr. Shiel received a Bachelor of Science degree with honors from the University of Notre Dame. There he was involved in research in radiation biology and received the Huisking Scholarship. After graduating from St. Louis University School of Medicine, he completed his Internal Medicine residency and Rheumatology fellowship at the University of California, Irvine. He is board-certified in Internal Medicine and Rheumatology.

Reviewed on 12/4/2018

Acrodermatitis enteropathica: An historic model for the therapy of genetic disease. In an era (the 1950s) when inherited disorders were usually seen as hopeless, this progressive hereditary (autosomal recessive) disease of children was found treatable. Acrodermatitis enteropathica is characterized by the simultaneous occurrence of skin inflammation (dermatitis) and diarrhea. The skin on the cheeks, elbows and knees and tissue about the mouth and anus are inflamed. There is also balding of the scalp, eyebrows and lashes, delayed wound healing and recurrent bacterial and fungal infections due to immune deficiency.

The key laboratory finding in acrodermatitis enteropathica is an abnormally low blood zinc level, reflecting impaired zinc uptake. Treatment with zinc by mouth is curative.

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Reviewed on 12/4/2018