Small intestine & ampulla
Malabsorption
Acrodermatitis enteropathica
Author: Hanni Gulwani, M.B.B.S.
Topic Completed: 1 August 2012
Minor changes: 18 November 2020
Copyright: 2003-2021, PathologyOutlines.com, Inc.
PubMed Search: Acrodermatitis enteropathica small bowel
Table of Contents
Definition / general | Treatment | Microscopic (histologic) description | Electron microscopy descriptionCite this page: Gulwani H. Acrodermatitis enteropathica. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/smallbowelacrodermatitis.html. Accessed December 8th, 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
