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Pediatric Acrodermatitis Enteropathica Treatment & Management

  • Author: KN Siva Subramanian, MD; Chief Editor: Dirk M Elston, MD  more...
 
Updated: Aug 18, 2016
 

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. See the following images.

Skin lesions in the diaper area. Skin lesions in the diaper area.
Skin lesions in the diaper area (see image above) Skin lesions in the diaper area (see image above) a few weeks after treatment with zinc.

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.

Substantial evidence for improvement with topical zinc application is not available.

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Consultations

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

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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 a new window)

Food Serving Size Zinc Content, mg
Oysters 6 medium, cooked 43.4
Dungeness crab 3 oz, cooked 4.6
Beef 3 oz, cooked 5.8
Turkey, dark meat 3 oz, cooked 3.5
Chicken, dark meat 3 oz, cooked 2.4
Pork 3 oz, cooked 2.2
Cashews 1 oz 1.6
Baked beans 0.5 cup 1.8
Yogurt, fruit 1 cup (8 oz) 1.8
Chickpeas (garbanzo beans) 0.5 cup 1.3
Almonds 1 cup (8 oz) 1.0
Milk 1 cup (8 oz) 1.0
Cheddar cheese 1 oz 0.9
Peanuts 1 cup (8 oz) 0.9

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

Life Stage Age Allowance, mg/d
Males Females
Infants 0-6 mo 2 2
7-12 mo 3 3
Children 1-3 y 3 3
4-8 y 5 5
9-13 y 8 8
Adolescents 14-18 y 11 9
Pregnant, ≤19 y NA 12
Breastfeeding, ≤19 y NA 13
Adults All, ≥19 y 11 8
Pregnant, ≥19 y NA 11
Breastfeeding, ≥19 y NA 12
NA = not applicable
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Complications

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

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Long-Term Monitoring

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

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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
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  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. Lee WJ, Kim CH, Won CH, Chang SE, Lee MW, Choi JH, et al. Bullous acrodermatitis enteropathica with interface dermatitis. J Cutan Pathol. 2010 Sep. 37 (9):1013-5. [Medline].

  14. Iyengar S, Chambers C, Sharon VR. Bullous acrodermatitis enteropathica: case report of a unique clinical presentation and review of the literature. Dermatol Online J. 2015 Apr 16. 21 (4):[Medline].

  15. 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. 2008 Oct. 35 Suppl 1:1-13. [Medline].

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

  17. Weinkle AP, Patel N, Kissel R, Seminario-Vidal L. Acquired acrodermatitis enteropathica as a presenting sign of celiac disease. JAAD Case Rep. 2016 May. 2 (3):193-5. [Medline].

  18. 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].

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

 
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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
Food Serving Size Zinc Content, mg
Oysters 6 medium, cooked 43.4
Dungeness crab 3 oz, cooked 4.6
Beef 3 oz, cooked 5.8
Turkey, dark meat 3 oz, cooked 3.5
Chicken, dark meat 3 oz, cooked 2.4
Pork 3 oz, cooked 2.2
Cashews 1 oz 1.6
Baked beans 0.5 cup 1.8
Yogurt, fruit 1 cup (8 oz) 1.8
Chickpeas (garbanzo beans) 0.5 cup 1.3
Almonds 1 cup (8 oz) 1.0
Milk 1 cup (8 oz) 1.0
Cheddar cheese 1 oz 0.9
Peanuts 1 cup (8 oz) 0.9
Table 2. Recommended Dietary Allowances for Zinc (elemental)
Life Stage Age Allowance, mg/d
Males Females
Infants 0-6 mo 2 2
7-12 mo 3 3
Children 1-3 y 3 3
4-8 y 5 5
9-13 y 8 8
Adolescents 14-18 y 11 9
Pregnant, ≤19 y NA 12
Breastfeeding, ≤19 y NA 13
Adults All, ≥19 y 11 8
Pregnant, ≥19 y NA 11
Breastfeeding, ≥19 y NA 12
NA = not applicable
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