Small intestine & ampulla
Malabsorption
Acrodermatitis enteropathica


Topic Completed: 1 August 2012

Minor changes: 18 November 2020

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PubMed Search: Acrodermatitis enteropathica small bowel

Hanni Gulwani, M.B.B.S.
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Cite this page: Gulwani H. Acrodermatitis enteropathica. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/smallbowelacrodermatitis.html. Accessed May 7th, 2021.
Definition / general
  • Autosomal recessive, linked to zinc deficiency, affects children
  • Genetic defect mapped to SLC39A4 gene at 8q24, encodes zinc transporter Zip4 (J Am Acad Dermatol 2007;56:116)
  • Cutaneous lesions (perioral and extremity skin lesions, alopecia, nail dystrophy), diarrhea, malabsorption
Treatment
  • Zinc sulfate
Microscopic (histologic) description
  • Severe villus abnormality in some; normal in others
Electron microscopy description
  • Rod-like fibrillar inclusions in Paneth cells
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