eMedicine Specialties > Dermatology > Metabolic Diseases

Acrodermatitis Enteropathica: Treatment & Medication

Author: Kristina Marie Dela Rosa, MD, Flight Surgeon, Commander Naval Air Forces, NAS North Island
Coauthor(s): Elizabeth Kline Satter, MD, MPH, Chairman, Department of Dermatology, Naval Medical Center San Diego
Contributor Information and Disclosures

Updated: Oct 30, 2009

Treatment

Medical Care

Treatment of acrodermatitis enteropathica requires lifelong zinc supplementation. Typically, 1-3 mg/kg of zinc gluconate or sulfate is administered orally each day.8,12 Clinical improvement occurs prior to any significant change in the plasma zinc levels, usually within days to weeks of initiating treatment. Monitor serum zinc levels and alkaline phosphatase values every 3-6 months.8

Acrodermatitis enteropathica exacerbation during pregnancy or the stress of disease may require an increase in therapy.1 26

Warm compresses to remove the scale crust, followed by application of white petrolatum to eroded skin lesions, may enhance reepithelialization when used concurrently with zinc replacement.

Diet

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 content.14,27

Activity

No activity restrictions are necessary for acrodermatitis enteropathica patients.

Medication

Mineral supplements

These agents are used to reduce morbidity and to prevent complications in acrodermatitis enteropathica.


Zinc gluconate (Verazinc, Zinca-Pak, Orazinc)

One 10-mg tab contains 1.4 mg of elemental zinc.

Adult

1 mg/kg/d PO for life

Pediatric

0.5-1 mg elemental zinc/kg PO qd, divided 1-3 times/d

May reduce effect of penicillamine, tetracycline, and quinolones; concomitant administration of copper or iron with zinc may decrease their gastrointestinal absorption

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; very high dosages can result in sideroblastic anemia and microcytic anemia, secondary to zinc-induced copper deficiency (latter can be associated with neurologic defects)

More on Acrodermatitis Enteropathica

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

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

  3. Kury S, Dreno B, Bezieau S, et al. Identification of SLC39A4, a gene involved in acrodermatitis enteropathica. Nat Genet. Jul 2002;31(3):239-40. [Medline].

  4. Nakano A, Nakano H, Nomura K, Toyomaki Y, Hanada K. Novel SLC39A4 mutations in acrodermatitis enteropathica. J Invest Dermatol. Jun 2003;120(6):963-6. [Medline].

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

  6. Schmitt S, Kury S, Giraud M, Dreno B, Kharfi M, Bezieau S. An update on mutations of the SLC39A4 gene in acrodermatitis enteropathica. Hum Mutat. Jun 2009;30(6):926-33. [Medline].

  7. Wang K, Zhou B, Kuo YM, Zemansky J, Gitschier J. A novel member of a zinc transporter family is defective in acrodermatitis enteropathica. Am J Hum Genet. Jul 2002;71(1):66-73. [Medline].

  8. Maverakis E, Fung MA, Lynch PJ, et al. Acrodermatitis enteropathica and an overview of zinc metabolism. J Am Acad Dermatol. Jan 2007;56(1):116-24. [Medline].

  9. Evans GW, Johnson PE. Zinc-binding factor in acrodermatitis enteropathica. Lancet. Dec 11 1976;2(7998):1310. [Medline].

  10. Samady JA, Schwartz RA, Shih LY, Piela Z, Lambert WC, Janniger CK. Acrodermatitis enteropathica-like eruption in an infant with nonketotic hyperglycinemia. J Dermatol. Sep 2000;27(9):604-8. [Medline].

  11. Aggett PJ, Atherton DJ, More J, Davey J, Delves HT, Harries JT. Symptomatic zinc deficiency in a breast-fed preterm infant. Arch Dis Child. Jul 1980;55(7):547-50. [Medline].

  12. 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].

  13. Connors TJ, Czarnecki DB, Haskett MI. Acquired zinc deficiency in a breast-fed premature infant. Arch Dermatol. Apr 1983;119(4):319-21. [Medline].

  14. Champion RH, Burton JL, Ebling FJG. Textbook of Dermatology. 3. London: Blackwell Science; 1998:2668.

  15. Roberts LJ, Shadwick CF, Bergstresser PR. Zinc deficiency in two full-term breast-fed infants. J Am Acad Dermatol. Feb 1987;16(2 Pt 1):301-4. [Medline].

  16. Zimmerman AW, Hambidge KM, Lepow ML, Greenberg RD, Stover ML, Casey CE. Acrodermatitis in breast-fed premature infants: evidence for a defect of mammary zinc secretion. Pediatrics. Feb 1982;69(2):176-83. [Medline].

  17. Schmidt CP, Tunnessen W. Cystic fibrosis presenting with periorificial dermatitis. J Am Acad Dermatol. Nov 1991;25(5 Pt 2):896-7. [Medline].

  18. Tabanlioglu D, Ersoy-Evans S, Karaduman A. Acrodermatitis enteropathica-like eruption in metabolic disorders: acrodermatitis dysmetabolica is proposed as a better term. Pediatr Dermatol. Mar-Apr 2009;26(2):150-4. [Medline].

  19. Niiyama S, Koelker S, Degen I, Hoffmann GF, Happle R, Hoffmann R. Acrodermatitis acidemica secondary to malnutrition in glutaric aciduria type I. Eur J Dermatol. May-Jun 2001;11(3):244-6. [Medline].

  20. Van Wouwe JP. Clinical and laboratory diagnosis of acrodermatitis enteropathica. Eur J Pediatr. Oct 1989;149(1):2-8. [Medline].

  21. Glover MT, Atherton DJ. Transient zinc deficiency in two full-term breast-fed siblings associated with low maternal breast milk zinc concentration. Pediatr Dermatol. Feb 1988;5(1):10-3. [Medline].

  22. Gonzalez JR, Botet MV, Sanchez JL. The histopathology of acrodermatitis enteropathica. Am J Dermatopathol. Aug 1982;4(4):303-11. [Medline].

  23. Mori H, Matsumoto Y, Tamada Y, Ohashi M. Apoptotic cell death in formation of vesicular skin lesions in patients with acquired zinc deficiency. J Cutan Pathol. Aug 1996;23(4):359-63. [Medline].

  24. Jensen SL, McCuaig C, Zembowicz A, Hurt MA. Bullous lesions in acrodermatitis enteropathica delaying diagnosis of zinc deficiency: a report of two cases and review of the literature. J Cutan Pathol. Oct 2008;35 Suppl 1:1-13. [Medline].

  25. Welsmann K, Kvist N, Kobayasi T. Bullous acrodermatitis due to zinc deficiency during total parenteral nutrition: an ultrastructural study of the epidermal changes. Acta Derm Venereol. 1983;63(2):143-6. [Medline].

  26. Perez-Maldonado A, Kurban AK. Metabolic Diseases and Pregnancy. In: Clinics in Dermatology. 24. Elsevier; 2006:88-90.

  27. Institute of Medicine, Food and Nutrition Board. Dietary Reference Intakes for Vitamin A, Vitamin K, Arsenic, Boron, Chromium, Copper, Iodine, Iron, Manganesse, Molybdenum, Nickel, Silicon, Vanadium, and Zinc. Available at http://ods.od.nih.gov/FactSheets/Zinc.asp#h3.. Accessed 9/9/09.

  28. Willis MS, Monaghan SA, Miller ML, et al. Zinc-induced copper deficiency: a report of three cases initially recognized on bone marrow examination. Am J Clin Pathol. Jan 2005;123(1):125-31. [Medline].

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Further Reading

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Keywords

acrodermatitis enteropathica, AE, zinc deficiency, congenital zinc deficiency

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Contributor Information and Disclosures

Author

Kristina Marie Dela Rosa, MD, Flight Surgeon, Commander Naval Air Forces, NAS North Island
Kristina Marie Dela Rosa, MD is a member of the following medical societies: American College of Physicians and American Medical Association
Disclosure: Nothing to disclose.

Coauthor(s)

Elizabeth Kline Satter, MD, MPH, Chairman, Department of Dermatology, Naval Medical Center San Diego
Elizabeth Kline Satter, MD, MPH is a member of the following medical societies: Alpha Omega Alpha and American Medical Women's Association
Disclosure: Nothing to disclose.

Medical Editor

Eleanor E Sahn, MD, Director, Division of Pediatric Dermatology, Associate Professor, Departments of Dermatology and Pediatrics, Medical University of South Carolina
Eleanor E Sahn, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, and Southern Medical Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center
Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Edward F Chan, MD, Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania School of Medicine
Edward F Chan, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.

CME Editor

Catherine M Quirk, MD, Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania
Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
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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