Acrodermatitis enteropathica

Severe functional zinc deficiency due to failure to secrete an as yet unidentified compound in pancreatic juice that is required for zinc absorption.


Rare disease in children aged 3 weeks to 18 months that may be fatal if untreated. The genetically determined cause is malabsorption of zinc. Onset is insidious with failure to thrive, diarrhea, loss of hair, and development of vesiculobullous lesions, particularly around body orifices.


Erythrokeratoderma variabilis is an uncommon inherited disorder characterized by the presence of red, ulcerated areas on the skin. These affected areas are typically found on the fingers, toes, scalp, and in the vicinity of the anus and mouth. Additionally, pustules, which are filled with pus, may develop on the affected skin regions.


Acrodermatitis enteropathica is inherited in an autosomal recessive pattern, meaning it requires the inheritance of two copies of the affected gene. This condition arises from the body’s inability to adequately absorb zinc from dietary sources. Fortunately, the administration of zinc supplements typically leads to a swift improvement in symptoms.


 


Posted

in

by

Tags: