Acrodermatitis Enteropathica Associated with Low Density Lipoproteins Deficiency

Abstract

This study describes clinical, biochemical, and histopathologic findings in a three-month-old infant with acrodermatitis enteropathica. Com plete absence of low density lipoprotein frac tions in serum was found on paper and cellogel electrophoresis. This is the first known patient with acrodermatitis enteropathica in whom a- beta-lipoproteinemia was diagnosed. Despite treatment with fluids, special diets, antibiotics and diiodoquine, the infant died after two months of hospitalization.. A marked reduction in the levels of pre-beta and beta-lipoproteins was also observed in the serum of a sister of the propositus, one month following birth, born after propositus' death. At present, the sister is six months old. During this period, a constant increase in the d < 1.063 lipoprotein fractions in the sister's serum was observed. The propositus and the entire family have been studied and results are presented.