Pediatric Acrodermatitis Enteropathica Differential Diagnoses

Updated: Sep 05, 2019
  • Author: KN Siva Subramanian, MD, FAAP; Chief Editor: Dirk M Elston, MD  more...
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Diagnostic Considerations

Acrodermatitis dysmetabolica (AD) is a new term used to describe the phenotypic appearance of other conditions that resemble acrodermatitis enteropathica (AE) but are associated with a normal plasma zinc level. Other than acquired zinc deficiency, most differential diagnoses are easily excluded. A successful trial of zinc supplementation confirms the diagnosis. However, if zinc supplementation does not improve the condition, AD should be considered. Biotin and zinc metabolism are closely linked. Both biotin deficiency and biotinidase deficiency may mimic acrodermatitis enteropathica. [20, 21]

The following should also be considered:

  • Acquired zinc deficiency of any cause

  • Infants receiving inadequate supplementation in parenteral alimentation

  • Infants who were born prematurely

  • Full-term or premature breastfed infants

  • Infants with malabsorption due to cystic fibrosis, small-bowel resection (especially those undergoing nasogastric decompression), or celiac disease [22]
  • Human immunodeficiency virus (HIV) disease

  • Atypical epidermolysis bullosa (AD)

  • Generalized or localized candidiasis (AD)

  • Abnormal metabolism of essential fatty acids (AD)

  • Seborrheic dermatitis

  • Kwashiorkor

  • Iatrogenic deficiency of branched chain amino acids (isoleucine) in restrictive diets for maple syrup urine disease, methylmalonic aciduria, or phenylketonuria (AD)

Differential Diagnoses