Acrodermatitis Enteropathica Clinical Presentation

Updated: Aug 21, 2019
  • Author: Kristina Marie Dela Rosa, MD; Chief Editor: William D James, MD  more...
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Presentation

History

Patients have a history of refractory diarrhea, failure to thrive, irritability, dermatitis, and alopecia that gradually appeared shortly after weaning from breast milk. Occasionally, patients have a history of siblings or other family members with similar symptoms in infancy. [1] Only 20% of cases have all the components of the triad of dermatitis, alopecia, and diarrhea. [1]

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Physical Examination

Physical signs and symptoms are as follows:

  • General: Infants are typically irritable and often inconsolable, and they show a slowing or cessation of growth and development.

  • Skin: Erythematous, dry, and scaly patches and plaques are present and may evolve into crusted, vesiculobullous, erosive, psoriasiform, and pustular lesions. Lesions are predominantly distributed in a periorificial and acral pattern and may become secondarily infected with Staphylococcus aureus or Candida albicans.

  • Mucosa: Findings include angular cheilitis, glossitis, conjunctivitis, blepharitis, punctate keratopathy, and photophobia.

  • Nails: Paronychia and nail dystrophy are typical.

  • Hair: Patients have loss of scalp hair, eyebrows, and eyelashes.

See the image below.

Sharply demarcated, brightly erythematous periorif Sharply demarcated, brightly erythematous periorificial plaque in an infant with acrodermatitis enteropathica.
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Complications

If untreated, the lesions of acrodermatitis enteropathica may become secondarily infected with Staphylococcus aureus and Candida albicans. Additionally, high-dose zinc supplementation occasionally causes gastric upset and can adversely affect copper metabolism. [21]

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