Zinc Therapy of Acrodermatitis Enteropathica

Abstract

The therapeutic effect of orally administered zinc was evaluated in an adult woman with acrodermatitis enteropathica. When she was off therapy and in clinical relapse the plasma zinc concentration (10 mug per 100 ml), serum alkaline phosphatase (3 1U per liter) and urine zinc excretion rate (39 mug per 24 hours) were extremely low. Di-iodohydroxyquin therapy was accompanied by a modest increase in plasma zinc concentrations. Oral zinc sulfate (220 mg three times a day or 50 mg twice a day) resulted in rapid and complete clinical remission, and in a return of plasma zinc, serum alkaline phosphatase and urinary zinc excretion to normal. These data are compatible with a severe zinc deficiency state and indicate that the inherited defect in this disease is either in or closely related to zinc metabolism. The beneficial effects of zinc therapy in this patient provide further confirmation of the efficacy of oral zinc in the treatment of acrodermatitis enteropathica.

Krüppel-like factor 4 regulates adaptive expression of the zinc transporterZip4in mouse small intestine

“…nutrient zinc transport; absorption; transcription; gene regulation SEVERAL MUTATIONS of the Slc39a4 gene (Zip4) produce acrodermatitis enteropathica in humans (23,33). Acrodermatitis enteropathica patients have low intestinal zinc absorption and severe zinc deficiency, including secondary cutaneous infections, as well as other manifestations of zinc deprivation (19,22,24,31). The demonstration that pathological signs abate in acrodermatitis enteropathica patients upon zinc supplementation (22,24,31) suggests that ZIP4 is a major zinc transporter in the gastrointestinal tract responsible for adequate zinc balance and homeostasis in humans.…”

“…Acrodermatitis enteropathica patients have low intestinal zinc absorption and severe zinc deficiency, including secondary cutaneous infections, as well as other manifestations of zinc deprivation (19,22,24,31). The demonstration that pathological signs abate in acrodermatitis enteropathica patients upon zinc supplementation (22,24,31) suggests that ZIP4 is a major zinc transporter in the gastrointestinal tract responsible for adequate zinc balance and homeostasis in humans. During zinc deficiency in rodents, zinc absorption increases primarily through increased kinetics of uptake by the intestine (12,20).…”

Serum Zinc and Copper Levels in Healthy Japanese Infants

“…Zinc and copper deficiencies in infants and children have been reported (Sturgeon and Hambidge et al 1976). Furthermore, acrodermatitis enteropathica has been described to be zinc-related syndrome (Moynahan 1974;Neldner and Hambidge 1975), and genetic abnormalities in copper absorption have been described in man (Menkes et al 1962;Yoshida et al 1969;Danks et al 1972). …”

A study on efficacy of oral zinc therapy for treatment of acrodermatitis enteropathica

“…Although no special diet is required for acrodermatitis enteropathica patients, as long as zinc supplementation is continued, certain foods contain increased levels of zinc, including oysters, crab, beef, pork, and fowl. Zinc content is directly related to protein [18,19].…”