Bullous presentation of Acrodermatitis Enteropathica in three female siblings: A case series
- Author:
Katherine Joy B. Sayo-Aguiling
1
;
Abelaine Venida-Tablizo
2
;
Melanie Joy D. Ruiz
1
Author Information
- Publication Type:Journal Article
- Keywords: Acrodermatitis enteropathica; Congenital zinc deficiency
- MeSH: Zinc; Blister
- From: Journal of the Philippine Medical Association 2024;102(2):95-104
- CountryPhilippines
- Language:English
- Abstract: Acrodermatitis enteropathica is a rare autosomal recessive disease that results from a defect in zinc metabolism. It is clinically characterized by a phenotypic triad of periorificial and acral dermatitis, diarrhea, and alopecia. Oral zinc therapy gives a rapid excellent clinical response and reduces mortality. We report three female pediatric siblings who presented with periorificial and acral dermatitis, diffuse alopecia, nail dystrophy, irritable mood, and stunted growth. A diagnosis of acrodermatitis enteropathicawas confirmed with markedly decreased levels of serum zinc. The patients were successfully treated with oral zinc sulfate at a dose of 5mg/kg/day for the first two weeks then maintained on a dose at 2mg/kg/day.
- Full text:2024060416331895634Bullous Presentation.pdf
