Acrodermatitis Enteropathica (AE) in Ophthalmology Treatment & Management

Updated: Jun 01, 2021
  • Author: John D Sheppard, Jr, MD, MMSc; Chief Editor: Hampton Roy, Sr, MD  more...
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Treatment

Medical Care

Systemic treatment for acrodermatitis enteropathica (AE) requires lifelong zinc supplementation. [16] Ocular therapy requires vigorous ocular surface supportive measures and intervention when secondary bacterial or candidal infection, trichiasis, or other complications occur. There is no known corrective genetic treatment available. Good copies of the defective gene have not yet been coupled to vectors capable of altering human phenotypes.

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Surgical Care

Advanced cases of AE may require oculoplastic surgical intervention including lid reconstruction, tarsorrhaphy, or epilation therapy. Ocular surface reconstruction with conjunctival transplantation or human amniotic membrane grafts also is a possibility in severe situations. Corneal transplantation may be required in cases of severe keratomalacia or infectious keratitis. These therapies are recommended with good judgment in the context of severe failure to thrive and limited predicted life span.

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Consultations

Consultation with a pediatrician, geneticist, dermatologist, and/or plastic surgeon may be useful.

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Diet

Treatment of AE involves greater than 1-2 mg/kg of oral zinc supplementation per day for life. No special diet is necessary as long as zinc supplementation is continued.

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Activity

No activity limitations are required in adequately treated patients. Patients with severe ocular-surface inflammation may require certain restrictions from dusty, dry, or dirty environments.

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Long-Term Monitoring

Long-term measures for AE include the following:

  • Lifelong zinc supplementation (>1-2 mg/kg of oral zinc supplementation per day)
  • Periodic ophthalmologic follow-up care, as indicated
  • Periodic dermatologic follow-up care, as indicated
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Further Inpatient Care

Severely ill infants with AE are admitted until stable. If untreated, cutaneous and lid lesions may become secondarily infected with S aureus and C albicans. Infants also may experience withdrawal, photophobia, and loss of appetite.

Further progression and even death from secondary infection may occur if AE is left untreated.

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Inpatient & Outpatient Medications

Inpatient and outpatient medications include the following:

  • Zinc supplementation (>1-2 mg/kg of oral zinc supplementation per day)
  • Topical ophthalmologic preparations for irritative or infectious complications, as indicated
  • Topical dermatologic preparations for irritative or infectious complications, as indicated
  • Systemic antibiotics for severe ocular or cutaneous infectious complications, as indicated
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