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.
Zinc ; Blister