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]

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: Although sharply demarcated eczematous and psoriasiform patches and plaques with peripheral scale and crust are the most common cutaneous findings, erosive, vesiculobullous, and pustular lesions also can occur.^{[28, 29]}Lesions are predominantly distributed in a periorificial (sparing the upper lip) 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 periorificial plaque in an infant with acrodermatitis enteropathica.

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.^[30]

Differential Diagnoses

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Media Gallery

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

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Author

Kristina Marie Dela Rosa, MD Dermatologist, Insight Dermatology, San Diego, CA

Kristina Marie Dela Rosa, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Elizabeth K Satter, MD, MPH Dermatologist and Dermatopathologist

Elizabeth K Satter, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Medical Women's Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Michael J Wells, MD, FAAD Dermatologic/Mohs Surgeon, The Surgery Center at Plano Dermatology

Michael J Wells, MD, FAAD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Texas Medical Association

Disclosure: Nothing to disclose.

Edward F Chan, MD Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania School of Medicine

Edward F Chan, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Chief Editor

William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

William D James, MD is a member of the following medical societies: American Academy of Dermatology, Society for Investigative Dermatology

Disclosure: Received income in an amount equal to or greater than $250 from: Elsevier; WebMD<br/>Served as a speaker for various universities, dermatology societies, and dermatology departments.

Additional Contributors

Eleanor E Sahn, MD Director, Division of Pediatric Dermatology, Associate Professor, Departments of Dermatology and Pediatrics, Medical University of South Carolina

Eleanor E Sahn, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Southern Medical Association

Disclosure: Nothing to disclose.

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, South Carolina Medical Association

Disclosure: Nothing to disclose.

Acknowledgements

The view(s) expressed herein are those of the authors and do not reflect the official policy or position of Naval Special Warfare Center, the U.S. Navy Medical Department, the U.S. Navy Office of the Surgeon General, the Department of the Navy, Department of Defense, or the U.S. Government.

Acrodermatitis Enteropathica Clinical Presentation

History

Physical Examination

Complications

References

Tables

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