Evidence for normal metabolism and interconversions of unsaturated fatty acids in acrodermatitis enteropathica

Acrodermatitis enteropathica is a rare disorder, presumably of genetic origin, characterized by cutaneous lesions, gastrointestinal abnormalities, and psychic disturbances. Several reports have considered acrodermatitis enteropathica to be a disorder of lipid metabolism, in particular of fatty acid interconversions of polyunsaturated fatty acids. Studies in three unrelated patients demonstrated normal distribution of fatty acids of individual plasma lipids and of erthrocyte phospholipids. Enzymatic activity of fatty acid interconversions in cultured skin fibroblasts was entirely normal.