Acrodermatitis Enteropathica Follow-up

  • Author: John D Sheppard Jr, MD, MMSc; Chief Editor: Hampton Roy Sr, MD   more...
 
Updated: Jun 8, 2009
 

Further Inpatient Care

  • Severely ill infants 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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Further Outpatient Care

  • Zinc supplementation as already described
  • Periodic ophthalmologic follow-up care as indicated
  • Periodic dermatologic follow-up care as indicated
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Inpatient & Outpatient Medications

  • Zinc supplementation as previously described
  • 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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Deterrence/Prevention

  • Genetic counseling may be important to parents inquiring about conceiving additional children. Additional family members at risk also may be discovered.
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Complications

  • Ophthalmologic irritative complications and secondary infections, particularly with Staphylococcus and C albicans
  • Lid disease including seborrheic and infectious blepharitis, trichiasis, and entropion
  • Severe corneal disease including ectasia, infectious keratitis, and keratomalacia
  • Secondary Sjögren syndrome and keratitis sicca
  • Dermatologic irritative states and secondary infections, particularly with Staphylococcus and C albicans
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Prognosis

  • With early diagnosis and zinc supplementation, the prognosis is good. Ophthalmic complications are far less severe when the systemic disease is treated. Advanced cases may have severe ophthalmic complications.
  • Ocular surface complications and infections
    • Blepharitis
    • Conjunctivitis
    • Keratitis, corneal ectasia, keratomalacia, and corneal neovascularization
  • Eyelid complications
    • Trichiasis and entropion with secondary corneal damage
    • Ectropion, ptosis, lash loss, brow loss, and other lid deformities
    • Symblepharon
  • Amblyopia
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Patient Education

  • Dietary and genetic counseling are important.
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Contributor Information and Disclosures
Author

John D Sheppard Jr, MD, MMSc  Professor of Ophthalmology, Microbiology and Molecular Biology, Clinical Director, Thomas R Lee Center for Ocular Pharmacology, Ophthalmology Residency Research Program Director, Eastern Virginia Medical School; President, Virginia Eye Consultants

John D Sheppard Jr, MD, MMSc is a member of the following medical societies: American Academy of Ophthalmology, American Society for Microbiology, American Society of Cataract and Refractive Surgery, American Uveitis Society, and Association for Research in Vision and Ophthalmology

Disclosure: Nothing to disclose.

Coauthor(s)

Timothy G Woodall, MD  Dermatology, Carolinas Medical Center - Pineville

Timothy G Woodall, MD is a member of the following medical societies: American Academy of Dermatology, American Society for Dermatologic Surgery, and South Carolina Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Andrew A Dahl, MD  Director of Ophthalmology Teaching, Mid-Hudson Family Practice Institute, The Institute for Family Health; Assistant Professor of Surgery (Ophthalmology), New York College of Medicine

Andrew A Dahl, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Ophthalmology, American College of Surgeons, American Medical Association, American Society of Cataract and Refractive Surgery, and Wilderness Medical Society

Disclosure: Nothing to disclose.

Simon K Law, MD, PharmD  Assistant Professor of Ophthalmology, Jules Stein Eye Institute; Chief of Section of Ophthalmology Surgical Services, Department of Veterans Affairs Healthcare Center, West Los Angeles

Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society, and Association for Research in Vision and Ophthalmology

Disclosure: Nothing to disclose.

Christopher J Rapuano, MD  Professor, Department of Ophthalmology, Jefferson Medical College of Thomas Jefferson University; Co-Chairman of the Cornea Service, Co-Chairman of Refractive Surgery Department, Wills Eye Institute

Christopher J Rapuano, MD is a member of the following medical societies: American Academy of Ophthalmology, American Society of Cataract and Refractive Surgery, Contact Lens Association of Ophthalmologists, Cornea Society, Eye Bank Association of America, International Society of Refractive Surgery, and Pan-American Association of Ophthalmology

Disclosure: Allergan Honoraria Speaking and teaching; Allergan Consulting fee Consulting; Alcon Honoraria Speaking and teaching; Inspire Honoraria Speaking and teaching; RPS Ownership interest Other; Vistakon Honoraria Speaking and teaching; EyeGate Pharma Consulting; Inspire Consulting fee Consulting; Bausch & Lomb Honoraria Speaking and teaching

Lance L Brown, OD, MD  Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri

Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD  Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

References

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