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acrodermatitis enteropathica
Etiology:
1) zinc deficiency
- Crohn's disease with or without ileal resection
- small intestinal disease [6]
2) inherited
3) acquired
Epidemiology: rare
Pathology:
- inability to absorb sufficient Zn+2
- immune system dysfunction
- skin & skin appendage pathology
Genetics:
- autosomal recessive
- associated with defects in SLC39A4
Clinical manifestations:
1) growth retardation
1) angular cheilitis
2) seborrheic dermatitis
3) skin erosions, blisters, pustules
4) erythematous vesiculobullous dermatitis especially involving
a) face
b) hands
c) feet
d) perineum
5) alopecia
6) diarrhea
7) occasionally mental disorders
* images [3,4,5]
Management:
1) zinc supplementation 3 mg/kg
2) without Zn+2 therapy, disease is fatal
Interactions
disease interactions
General
acrodermatitis
disorder of zinc metabolism
genetic disease of the gastrointestinal tract
genetic disease of the skin (genodermatosis)
Database Correlations
OMIM 201100
References
- Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed)
Lippincott-Raven, Philadelphia, 1998, pg 174
- OMIM :accession 201100
- DermNet NZ. Acrodermatitis enteropathica (images)
http://www.dermnetnz.org/systemic/acrodermatitis-enteropathica.html
- Sivakumar A, Vageshappa RK, Kumari R.
Acrodermatitis Enteropathica.
JAMA Dermatol. Published online November 8, 2023
PMID: 37938848
https://jamanetwork.com/journals/jamadermatology/fullarticle/2811789
- Guliani A, Bishnoi A.
Acquired Acrodermatitis Enteropathica.
JAMA Dermatol. 2019 Nov 1;155(11):1305.
PMID: 31461117
https://jamanetwork.com/journals/jamadermatology/fullarticle/2747827
- NEJM Knowledge+