Pediatric Acrodermatitis Enteropathica Workup

  • Author: KN Siva Subramanian, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Aug 16, 2010
 

Laboratory Studies

  • In patients with acrodermatitis enteropathica (AE), the zinc concentration of plasma is measured. Specimens are collected in plastic syringes or acid-washed Vacutainer tubes to prevent exogenous contamination that could lead to spuriously normal measurements.
    • In most patients with AE, plasma zinc concentrations are low (< 50 mcg/dL) but are not diagnostic.
    • Zinc concentrations within the reference range have been reported in patients with AE, and low zinc concentrations have been reported in patients without AE.
    • Most of the zinc accretion in a fetus occurs during the third trimester (at a rate of 850 mcg/kg/d). Therefore, in premature infants, a lack of stored zinc may precipitate symptoms early, especially if they are fed with formula.
  • Hair, saliva, or urine zinc levels are rarely needed.
  • Production of serum alkaline phosphatase depends on zinc; therefore, a low level of alkaline phosphatase may support a diagnosis of AE.
  • Secondary infections may require cultures and additional therapy.
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Procedures

  • Skin or intestinal mucosal biopsy is rarely needed.
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Histologic Findings

  • Intestinal mucosal biopsy reveals a loss of villous architecture with increased cell infiltration in the lamina propria in patients with AE. The nuclei are enlarged with an open chromatin distribution.
  • Complete normalization of the intestinal mucosa is observed in mucosal biopsy samples after zinc sulphate treatment.
  • Histopathology of cutaneous lesions reveals intracellular edema and pallor of the upper third of the epidermis. This finding is not pathognomonic and may be observed in other states of nutritional deficiency.
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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, 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 College of Nutrition, American Society for Parenteral and Enteral Nutrition, American Society of Law, Medicine & 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  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 and American Medical Association

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.

Specialty Editor Board

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.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Pharmacy Editor, eMedicine

Disclosure: Nothing to disclose.

Robert A Schwartz, MD, MPH  Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and 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.

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.

References

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  4. 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. Jul 2009;161(1):184-6. [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
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
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