eMedicine Specialties > Pediatrics: General Medicine > Dermatology

Acrodermatitis Enteropathica: Treatment & Medication

Author: KN Siva Subramanian, MD, Professor of Pediatrics and Obstetrics/Gynecology, Chief of Neonatology, Director of Nurseries, Georgetown University Medical Center
Coauthor(s): Robert A Silverman, MD, Clinical Associate Professor, Department of Pediatrics, Georgetown University; Clinical Associate Professor, Departments of Pediatrics and Dermatology, University of Virginia at Charlottesville; Aimee M Barton, MD, Fellow in Neonatal-Perinatal Medicine, Department of Pediatrics, Division of Neonatology, Georgetown University Medical Center; Sepideh Montazami, MD, Assistant Professor, Department of Pediatrics, Section of Neonatal-Perinatal Medicine, Georgetown University School of Medicine
Contributor Information and Disclosures

Updated: Sep 29, 2008

Treatment

Medical Care

  • In patients with acrodermatitis enteropathica (AE), zinc gluconate or sulfate is administered orally at a dosage of 1-3 mg/kg/d. Although the intravenous dosage has not clearly been estimated, amounts of 300-1000 mcg/kg/d may be sufficient for rapid reversal of symptoms.
    • Clinical response is observed within 5-10 days.
    • In AE, maintain zinc therapy throughout the patient's life span, though periods of remission are reported.
    • Exacerbation during pregnancy or the stress of disease may require an increase in therapy.
    • In acquired zinc deficiency, treatment can be stopped after the precipitating cause is resolved.
  • Warm compresses and petrolatum applied 3 times a day to areas of weeping or crusted dermatitis may enhance re-epithelialization when used concurrently with zinc replacement.

Consultations

  • Consultation with pediatricians, dermatologists, pediatric gastroenterologists, and/or nutritionists may be necessary.

Diet

  • The zinc content of some relatively zinc-rich foods is listed in Table 1 below.

    Table 1. Zinc Content of Zinc-Rich Foods
  • Open table in new window

    Table
    FoodServing SizeZinc Content, mg
    Oysters6 medium, cooked43.4
    Dungeness crab3 oz, cooked4.6
    Beef3 oz, cooked5.8
    Turkey, dark meat3 oz, cooked3.5
    Chicken, dark meat3 oz, cooked2.4
    Pork3 oz, cooked2.2
    Cashews1 oz1.6
    Baked beans0.5 cup1.8
    Yogurt, fruit1 cup (8 oz)1.8
    Chickpeas (garbanzo beans)0.5 cup1.3
    Almonds1 cup (8 oz)1.0
    Milk1 cup (8 oz)1.0
    Cheese cheddar1 oz0.9
    Peanuts1 cup (8 oz)0.9
    FoodServing SizeZinc Content, mg
    Oysters6 medium, cooked43.4
    Dungeness crab3 oz, cooked4.6
    Beef3 oz, cooked5.8
    Turkey, dark meat3 oz, cooked3.5
    Chicken, dark meat3 oz, cooked2.4
    Pork3 oz, cooked2.2
    Cashews1 oz1.6
    Baked beans0.5 cup1.8
    Yogurt, fruit1 cup (8 oz)1.8
    Chickpeas (garbanzo beans)0.5 cup1.3
    Almonds1 cup (8 oz)1.0
    Milk1 cup (8 oz)1.0
    Cheese cheddar1 oz0.9
    Peanuts1 cup (8 oz)0.9

    Table 2. Recommended Dietary Allowances for Zinc (elemental)
  • Open table in new window

    Table
    Life StageAgeAllowance, mg/d
    MalesFemales
    Infants0-6 mo22
    7-12 mo33
    Children1-3 y33
    4-8 y55
    9-13 y88
    Adolescents14-18 y119
    Pregnant, £ 19 yNA12
    Breastfeeding, £ 19 yNA13
    AdultsAll, ³ 19 y118
    Pregnant, ³ 19 yNA11
    Breastfeeding, ³ 19 yNA12
    Life StageAgeAllowance, mg/d
    MalesFemales
    Infants0-6 mo22
    7-12 mo33
    Children1-3 y33
    4-8 y55
    9-13 y88
    Adolescents14-18 y119
    Pregnant, £ 19 yNA12
    Breastfeeding, £ 19 yNA13
    AdultsAll, ³ 19 y118
    Pregnant, ³ 19 yNA11
    Breastfeeding, ³ 19 yNA12

    NA = not applicable.

Medication

Zinc supplements

Zinc is essential to normal growth and tissue repair and is important to protein and carbohydrate metabolism.


Zinc sulfate or gluconate (Verazinc, Zinca-Pak, Orazinc)

Cofactor for >70 types of enzymes.
Plays a role in many metabolic processes.
Elemental zinc 1 mg = zinc sulfate 4.4 mg. Elemental zinc 1 mg = zinc gluconate 7.1 mg.

Adult

Sulfate or gluconate salts: 1-3 mg/kg/d PO; alternatively 220 mg (as sulfate) PO tid

Pediatric

Sulfate or gluconate salts: 1-3 mg/kg/d PO

May reduce fluoroquinolones, penicillamine and tetracycline (except doxycycline) bioavailability; some foods (eg, bran, protein) decrease zinc bioavailability

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Caution in renal impairment; gastric upset observed with high doses; long-term high doses may decrease levels of high-density lipoprotein cholesterol (HDL-C) and decrease function of lymphocytes and polymorphonuclear (PMN) cells

Skin protectants

These protectants may enhance reepithelialization when used concurrently with zinc replacement.


Petrolatum (Vaseline)

Provides relief of minor skin irritations.

Adult

Apply to affected areas tid

Pediatric

Apply as in adults

Pregnancy

A - Fetal risk not revealed in controlled studies in humans

Precautions

For external use only; do not apply to eyes

More on Acrodermatitis Enteropathica

Overview: Acrodermatitis Enteropathica
Differential Diagnoses & Workup: Acrodermatitis Enteropathica
Treatment & Medication: Acrodermatitis Enteropathica
Follow-up: Acrodermatitis Enteropathica
Multimedia: Acrodermatitis Enteropathica
References

References

  1. Wang K, Pugh EW, Griffen S, et al. Homozygosity mapping places the acrodermatitis enteropathica gene on chromosomal region 8q24.3. Am J Hum Genet. Apr 2001;68(4):1055-60. [Medline].

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  3. Wang S, Xue L, Guo ZP, Wang L, Yang Y. A novel SLC39A4 gene mutation in the family of an acrodermatitis enteropathica patient with an unusual presentation. Br J Dermatol. Aug 5 2008;[Medline].

  4. Prasad AS. Zinc: an overview. Nutrition. Jan-Feb 1995;11(1 Suppl):93-9. [Medline].

  5. Kiechl-Kohlendorfer U, Fink FM, Steichen-Gersdorf E. Transient symptomatic zinc deficiency in a breast-fed preterm infant. Pediatr Dermatol. Sep-Oct 2007;24(5):536-40. [Medline].

  6. Wells BT, Winkelmann RD. Acrodermatitis enteropathica. Report of 6 cases. Arch Dermatol. Jul 1961;84:40-52. [Medline].

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  8. Barnes PM, Moynahan EJ. Zinc deficiency in acrodermatitis enteropathica: multiple dietary intolerance treated with synthetic diet. Proc R Soc Med. Apr 1973;66(4):327-9. [Medline].

  9. Bilinski DL, Ehrenkranz RA, Cooley-Jacobs J, et al. Symptomatic zinc deficiency in a breast-fed, premature infant. Arch Dermatol. Sep 1987;123(9):1221-4. [Medline].

  10. Chimenti F, Aouffen M, Favier A. Zinc homeostasis-proteins: new drug targets for triggering cell fate. Curr Drug Targets. 2003;4:323-8.

  11. Dallaha CJ, Lorincz AL, Aarnik ON. Acrodermatitis enteropathica: Review of the literature and report on a case successfully treated with diodoquin. JAMA. 1953;152:509-12.

  12. Danbolt N, Closs K. Acrodermatitis enteropathica. Acta Derm Venerol. 1942;22:17.

  13. Food and Nutrition Board, Institute of Medicine. Dietary reference intakes for vitamin A, vitamin K, boron, chromium, copper, iodine, iron, manganese, molybdenum, nickel, silicon, vanadium, and zinc. Washington, DC: National Academy Press; 2001:442-501. [Full Text].

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  15. Grider A, Mouat MF. The acrodermatitis enteropathica mutation affects protein expression in human fibroblasts: analysis by two-dimensional gel electrophoresis. J Nutr. Aug 1998;128(8):1311-4. [Medline].

  16. Hambidge KM. The role of zinc and other trace metals in pediatric nutrition and health. Pediatr Clin North Am. Feb 1977;24(1):95-106. [Medline].

  17. Hurley LS, Eckhert CD, Duncan JR, et al. [Acrodermatitis enteropathica and human breast milk]. Lancet. Jan 22 1977;1(8004):195. [Medline].

  18. Kharfi M, Zaraa I, Kury S, et al. [Acrodermatitis enteropathica in full-term breast-fed infant]. Ann Dermatol Venereol. Mar 2005;132(3):246-8. [Medline].

  19. Kuramoto Y, Igarashi Y, Tagami H. Acquired zinc deficiency in breast-fed infants. Semin Dermatol. Dec 1991;10(4):309-12. [Medline].

  20. Lonnerdal B, Stanislowski AG, Hurley LS. Isolation of a low molecular weight zinc binding ligand from human milk. J Inorg Biochem. Jan 1980;12(1):71-8. [Medline].

  21. Martin DP, Tangsinmankong N, Sleasman JW, et al. Acrodermatitis enteropathica-like eruption and food allergy. Ann Allergy Asthma Immunol. Mar 2005;94(3):398-401. [Medline].

  22. Moynahan EJ. Letter: Acrodermatitis enteropathica: a lethal inherited human zinc-deficiency disorder. Lancet. Aug 17 1974;2(7877):399-400. [Medline].

  23. Moynahan EJ, Barnes PM. Zinc deficiency and a synthetic diet for lactose intolerance. Lancet. Mar 24 1973;1(7804):676-7. [Medline].

  24. Piela Z, Szuber M, Mach B, et al. Zinc deficiency in exclusively breast-fed infants. Cutis. Apr 1998;61(4):197-200. [Medline].

  25. Portnoy B, Molokhia M. Acrodermatitis enteropathica treated by zinc. Br J Dermatol. Dec 1974;91(6):701-3. [Medline].

  26. Samady JA, Schwartz RA, Shih LY, et al. Acrodermatitis enteropathica-like eruption in an infant with nonketotic hyperglycinemia. J Dermatol. Sep 2000;27(9):604-8. [Medline].

  27. Sivasubramanian KN, Henkin RI. Behavioral and dermatologic changes and low serum zinc and copper concentrations in two premature infants after parenteral alimentation. J Pediatr. Nov 1978;93(5):847-51. [Medline].

  28. Sivasubramanian KN, Hoy G, Davitt MK, et al. Zinc and copper changes after neonatal parenteral alimentation. Lancet. Mar 4 1978;1(8062):508. [Medline].

  29. Walravens PA, Hambidge KM, Neldner KH, et al. Zinc metabolism in acrodermatitis enteropathica. J Pediatr. Jul 1978;93(1):71-3. [Medline].

Further Reading

Keywords

acrodermatitis enteropathica, AE, skin inflammation, periorificial dermatitis, acral dermatitis, alopecia, error of zinc metabolism, zinc deficiency, periorificial dermatitis, acral dermatitis, diarrhea, failure to thrive, anorexia, alopecia, nail dystrophy, malabsorption, geophagia, food allergy, growth retardation, photophobia, paronychia

Contributor Information and Disclosures

Author

KN Siva Subramanian, MD, Professor of Pediatrics and Obstetrics/Gynecology, Chief of Neonatology, Director of Nurseries, Georgetown University Medical Center
KN Siva Subramanian, MD is a member of the following medical societies: American Academy of Pediatrics, American Association for the Advancement of Science, American College of Nutrition, American Society for Parenteral and Enteral Nutrition, American Society of Law Medicine and Ethics, New York Academy of Sciences, and Southern Society for Pediatric Research
Disclosure: Nothing to disclose.

Coauthor(s)

Robert A Silverman, MD, Clinical Associate Professor, Department of Pediatrics, Georgetown University; Clinical Associate Professor, Departments of Pediatrics and Dermatology, University of Virginia at Charlottesville
Robert A Silverman, MD is a member of the following medical societies: American Academy of Dermatology and American Academy of Pediatrics
Disclosure: Nothing to disclose.

Aimee M Barton, MD, Fellow in Neonatal-Perinatal Medicine, Department of Pediatrics, Division of Neonatology, Georgetown University Medical Center
Aimee M Barton, MD is a member of the following medical societies: American Academy of Pediatrics, American Medical Association, and Medical Society of Virginia
Disclosure: Nothing to disclose.

Sepideh Montazami, MD, Assistant Professor, Department of Pediatrics, Section of Neonatal-Perinatal Medicine, Georgetown University School of Medicine
Sepideh Montazami, MD is a member of the following medical societies: American Academy of Pediatrics, American Medical Association, British Medical Association, and Royal College of Physicians
Disclosure: Nothing to disclose.

Medical Editor

Kevin P Connelly, DO, Clinical Assistant Professor, Department of Pediatrics, Division of General Pediatrics and Emergency Care, Virginia Commonwealth University; Medical Director, Paws for Health Pet Visitation Program of the Richmond SPCA; Pediatric Emergency Physician, Emergency Consultants Inc, Chippenham Medical Center
Kevin P Connelly, DO is a member of the following medical societies: American Academy of Pediatrics, American College of Osteopathic Pediatricians, and American Osteopathic Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

Robert A Schwartz, MD, MPH, Professor and Head of Dermatology, Professor of Medicine, Professor of Pediatrics, Professor of Pathology, Professor of Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi
Disclosure: Nothing to disclose.

CME Editor

Merrily P M Poth, MD, Professor, Department of Pediatrics and Neuroscience, Uniformed Services University of the Health Sciences
Merrily P M Poth, MD is a member of the following medical societies: American Academy of Pediatrics, Endocrine Society, and Lawson-Wilkins Pediatric Endocrine Society
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

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