ACRODERMATITIS ENTEROPATHICA - ABNORMALITIES OF FAT-METABOLISM AND INTEGUMENTAL ULTRASTRUCTURES IN INFANTS

Acrodermatitis enteropathica is a rare disease of childhood, characterized by periorificial and perioral dermatitis, acrodermatitis, diarrhea and alopecia. Two patients were successfully treated with diiodohydroxyquin (Diodoquin). Lipid concentrations disclosed abnormal distribution of fats that resembled those of less mature infants fed diets low in linoleic acid, even though the patients were actually on diets with high linoleic acid levels. Fatty acid distribution showed increased levels of linoleic acid that correlated with clinical improvement in the patient who was more ill. Skin fatty acids showed decreased levels of linoleic acid. Electron micrographs and histochemical staining disclosed increased lipid droplets in epidermal cells. Linoleic acid and zinc may have a role in the origin of the disease, and measurement of both skin and serum linoleic acid is suggested in the evaluation of its progress.