Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Pediatric Acrodermatitis Enteropathica Follow-up

  • Author: KN Siva Subramanian, MD; Chief Editor: Dirk M Elston, MD  more...
 
Updated: Aug 21, 2014
 

Further Outpatient Care

See the list below:

  • In infants and children with acrodermatitis enteropathica (AE), outpatient follow-up care is critical to ensure proper growth and development.
Next

Inpatient & Outpatient Medications

See the list below:

  • Zinc gluconate or sulfate therapy is lifelong for patients with AE.
Previous
Next

Complications

See the list below:

  • High-dose zinc supplementation may cause gastric upset.
  • High-dose zinc supplementation may also adversely affect copper metabolism.
Previous
Next

Prognosis

See the list below:

  • Patients with AE uniformly respond to zinc therapy with a 100% survival rate.
  • With zinc supplementation, various symptoms completely resolve or substantially improve.
  • In a noncompliant patient, many of the described complications (including death) can occur.
Previous
Next

Patient Education

See the list below:

  • Provide information regarding zinc deficiency as the cause of AE.
  • Emphasize the importance of lifelong compliance in taking zinc supplements.
Previous
 
Contributor Information and Disclosures
Author

KN Siva Subramanian, MD Professor of Pediatrics and Obstetrics/Gynecology, Chief of Neonatal Perinatal Medicine, Hospital Ethicist, Medstar Georgetown University Hospital

KN Siva Subramanian, MD is a member of the following medical societies: American Academy of Pediatrics, American Association for the Advancement of Science, American Society for Parenteral and Enteral Nutrition, New York Academy of Sciences, American College of Nutrition, American Society of Law, Medicine & Ethics, Southern Society for Pediatric Research

Disclosure: Nothing to disclose.

Coauthor(s)

Aimee M Barton, MD Assistant Professor, 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

Disclosure: Nothing to disclose.

Sepideh Montazami, MD Assistant Professor, Department of Pediatrics, Division 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, Royal College of Physicians

Disclosure: Nothing to disclose.

Dominique C Pichard, MD, MS Faculty, Department of Dermatology, Children's National Medical Center

Dominique C Pichard, MD, MS is a member of the following medical societies: International Society of Dermatology, Society for Pediatric Dermatology, Women's Dermatologic Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public Affairs and Administration

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, New York Academy of Medicine, American Academy of Dermatology, American College of Physicians, Sigma Xi

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Kevin P Connelly, DO Clinical Assistant Professor, Department of Pediatrics, Division of General Pediatrics and Emergency Care, Virginia Commonwealth University School of Medicine; 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, American Osteopathic Association

Disclosure: Nothing to disclose.

Acknowledgements

Robert A Silverman, MD Clinical Associate Professor, Department of Pediatrics, Georgetown University Medical Center

Robert A Silverman, MD is a member of the following medical societies: American Academy of Dermatology and Society for Pediatric Dermatology

Disclosure: Nothing to disclose.

References
  1. Kumar P, Lal NR, Mondal AK, Mondal A, Gharami RC, Maiti A. Zinc and skin: a brief summary. Dermatol Online J. 2012 Mar 15. 18(3):1. [Medline].

  2. Kawamura T, Ogawa Y, Nakamura Y, Nakamizo S, Ohta Y, Nakano H. Severe dermatitis with loss of epidermal Langerhans cells in human and mouse zinc deficiency. J Clin Invest. 2012 Feb 1. 122(2):722-32. [Medline].

  3. Eide DJ. The SLC39 family of metal ion transporters. Pflugers Arch. 2004 Feb. 447(5):796-800. [Medline].

  4. 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. 2008 Aug 5. [Medline].

  5. Nakano H, Nakamura Y, Kawamura T, et al. Novel and recurrent nonsense mutation of the SLC39A4 gene in Japanese patients with acrodermatitis enteropathica. Br J Dermatol. 2009 Jul. 161(1):184-6. [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. 2009 Jun. 30(6):926-33. [Medline].

  7. Murthy SC, Udagani MM, Badakali AV, Yelameli BC. Symptomatic zinc deficiency in a full-term breast-fed infant. Dermatol Online J. 2010 Jun 15. 16(6):3. [Medline].

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

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

  10. Corbo MD, Lam J. Zinc deficiency and its management in the pediatric population: a literature review and proposed etiologic classification. J Am Acad Dermatol. 2013 Oct. 69(4):616-624.e1. [Medline].

  11. Macdonald JB, Connolly SM, DiCaudo DJ. Think zinc deficiency: acquired acrodermatitis enteropathica due to poor diet and common medications. Arch Dermatol. 2012 Aug. 148(8):961-3. [Medline].

  12. Krebs NF. Update on zinc deficiency and excess in clinical pediatric practice. Ann Nutr Metab. 2013. 62 Suppl 1:19-29. [Medline].

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

  14. Arakawa T, Tamura T, Igarashi Y, et al. Zinc deficiency in two infants during total parenteral alimentation for diarrhea. Am J Clin Nutr. 1976 Feb. 29(2):197-204. [Medline].

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

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

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

  18. 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.

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

  20. 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].

  21. Graves K, Kestenbaum T, Kalivas J. Hereditary acrodermatitis enteropathica in an adult. Arch Dermatol. 1980 May. 116(5):562-4. [Medline].

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

Previous
Next
 
Skin lesions in the diaper area.
Skin lesions in the diaper area (see image above) a few weeks after treatment with zinc.
Facial lesions.
Foot lesions.
Table 1. Zinc Content of Zinc-Rich Foods
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)
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, ≥ 19 y118
Pregnant, ≥ 19 yNA11
Breastfeeding, ≥ 19 yNA12
NA = not applicable
Previous
Next
 
 
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.