Abstract
Acrodermatitis Enteropathica(AE), a rare autosomal recessive disorder is characterized by onset before age 1,failure to thrive diarrhea,alopecia,a vesciculobullous eruption around the mouth, anus hands and feet,high susceptibility to infection.The disorder is related to zinc metabolism and low level of plasma zinc have been reported in these patients.A cellular chemotaxis defect zinc dependent has been demonstrated by Weston et al.(Arch.Dermatol. 1977).We have investigated a 5 month old infant who developed the clinical features of AE when weaned from breast milk to cow's milk. Serum zinc concentration were 50μg%.The evaluation of the immune system revealed a normal T and B lymphocyte functions but a severe defect of neutrophil chemotaxis was present.The therapy with zinc sulphate 40mg/os/daily induced a dramatical clinical improvement, the restoration of plasma zinc level and the correction of neutrophil cellular chemotaxis.When the zinc therapy was discontinued cellular chemotaxis decreased(Tab.).
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Businco, L., Menghi, A., Rossi, P. et al. Zinc therapy in an infant with Acrodermatitis Enteropathica: clinical and imnunological result: 86. Pediatr Res 14, 179 (1980). https://doi.org/10.1203/00006450-198002000-00113
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DOI: https://doi.org/10.1203/00006450-198002000-00113