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Acrodermatitis Enteropathica

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Definition

An historic model for Acrodermatitis enteropathica: An historic model for thetherapy of genetic disease. In an era (the 1950s) when inherited disorders were usuallyseen as hopeless, this progressive hereditary (autosomal recessive) disease of childrenwas found treatable. Acrodermatitis enteropathica is characterized by the simultaneousoccurrence of skin inflammation (dermatitis) and diarrhea. The skin on the cheeks, elbowsand knees and tissue about the mouth and anus are inflamed. There is also balding of thescalp, eyebrows and lashes, delayed wound healing and recurrent bacterial and fungalinfections 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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