acrodermatitis enteropathica
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Etiology
- zinc deficiency
- Crohn's disease with or without ileal resection
- small intestinal disease[6]
- inherited
- 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
- growth retardation
- angular cheilitis
- seborrheic dermatitis
- skin erosions, blisters, pustules
- erythematous vesiculobullous dermatitis especially involving
- face
- hands
- feet
- perineum
- alopecia
- diarrhea
- occasionally mental disorders
Management
- zinc supplementation 3 mg/kg
- without Zn+2 therapy, disease is fatal
More general terms
- acrodermatitis
- genetic disease of the gastrointestinal tract
- genetic disease of the skin (genodermatosis)
- disorder of zinc metabolism
References
- ↑ Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 174
- ↑ OMIM https://mirror.omim.org/entry/201100
- ↑ 3.0 3.1 DermNet NZ. Acrodermatitis enteropathica (images) http://www.dermnetnz.org/systemic/acrodermatitis-enteropathica.html
- ↑ 4.0 4.1 Sivakumar A, Vageshappa RK, Kumari R. Acrodermatitis Enteropathica. JAMA Dermatol. Published online November 8, 2023 PMID: https://www.ncbi.nlm.nih.gov/pubmed/37938848 https://jamanetwork.com/journals/jamadermatology/fullarticle/2811789
- ↑ 5.0 5.1 Guliani A, Bishnoi A. Acquired Acrodermatitis Enteropathica. JAMA Dermatol. 2019 Nov 1;155(11):1305. PMID: https://www.ncbi.nlm.nih.gov/pubmed/31461117 https://jamanetwork.com/journals/jamadermatology/fullarticle/2747827
- ↑ 6.0 6.1 NEJM Knowledge+