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Acrodermatitis Enteropathica Associated With Inflammatory Bowel Disease

Weissman, Simcha DO1; Aziz, Muhammad MD2; Saleem, Saad MD3; Hassan, Ammar DO4; Sciarra, Michael DO4

doi: 10.14309/crj.0000000000000209

1Department of Medicine, Hackensack University (Palisades) Medical Center, North Bergen, NJ

2Department of Medicine, University of Kansas Medical Center, Kansas City, MO

3Department of Medicine, Mercy St. Vincent Medical Center, Toledo, OH

4Division of Gastroenterology, Hackensack University (Palisades) Medical Center, North Bergen, NJ

Correspondence: Simcha Weissman, DO, Department of Medicine, Hackensack University (Palisades) Medical Center, 7600 River Rd, North Bergen, NJ 07047 (

Received February 21, 2019

Accepted August 02, 2019

Online date: August 29, 2019

This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal.

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A 27-year-old male with a history of ileocolonic Crohn's disease (CD) status-post recent flare-up manifesting as abdominal pain and watery bowel movements presented with new-onset pruritic lesions. Physical examination revealed a scaly, dry, inflamed, and eczematous-appearing rash, located bilaterally on the shins (Figure 1). Of note, the patient has also recently noticed diarrhea (since the resolution of his flare-up) and new-onset baldness. Laboratory data showed low serum levels of zinc (19 μmol/dL) as well as low normal serum alkaline phosphatases (53 U/L). He was started on oral zinc sulfate therapy 3 mg/kg/d for 14 days, with a clinical suspicion of acrodermatitis enteropathica, a rare extraintestinal manifestation of CD. He demonstrated symptomatic improvement within 2 weeks of treatment and was continued on 1 mg/kg/d thereafter.

Figure 1

Figure 1

Extraintestinal manifestations occur in up to as many as 40% of patients with inflammatory bowel disease, and the prevalence is higher in CD compared to ulcerative colitis.1 Uveitis, pyoderma gangrenosum, erythema nodosum, and apthous stomatitis are among the more common manifestations reported. Acrodermatitis enteropathica is generally caused by an autosomal recessive mutation of the SLC39A4 gene located on chromosome 8q24.3, which is the gene responsible for zinc transportation.2 Being hereditary in nature, almost all cases occur in the pediatric population, causing malabsorption of the essential mineral zinc.3 Although rare, acrodermatitis enteropathica may also be seen in adults, because of conditions that cause intestinal malabsorption of zinc, such as our case.4

Clinically acrodermatitis enteropathica is characterized by the presence of red and inflamed areas of dry skin that evolve into pus-containing lesions. A diffuse loss of hair on the scalp, oral ulcers, and diarrhea are other common manifestations.5 The diagnosis can be suggested if low serum levels of zinc or alkaline phosphatase—a zinc-dependent enzyme—are detected, in combination with the clinical symptoms. The diagnosis can then be confirmed by a rapid clinical response to zinc supplementation or via skin biopsy. Although rare, AE should now be considered in patients with inflammatory bowel disease with supporting laboratory data and skin lesions of an unknown etiology.

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Author contributions: S. Weissman, M. Aziz, and S. Saleem wrote the manuscript and reviewed the literature. A. Hassan and M. Sciarra edited the manuscript. S. Weissman is the article guarantor.

Financial disclosure: None to report.

Informed consent was obtained for this case report.

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2. Küry S, Dréno B, Bézieau S, et al. Identification of SLC39A4, a gene involved in acrodermatitis enteropathica. Nat Genet. 2002;31(3):239–40.
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5. Cheshire H, Stather P, Vorster J. Acquired acrodermatitis enteropathica due to zinc deficiency in a patient with pre-existing Darier's disease. J Dermatol Case Rep. 2009;3(3):41–3.
© 2019 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of The American College of Gastroenterology.