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A case of acquired acrodermatitis enteropathica with a normal serum zinc level but a low level in the hair

Korean Journal of Pediatrics 2007;50(2):209-212.
Published online February 15, 2007.
A case of acquired acrodermatitis enteropathica with a normal serum zinc level but a low level in the hair
Kyung Il Oh, Jung Hee Kim, Ji Eun Lee, Dae Hyun Lim, Byong Kwan Son
Department of Pediatrics, College of Medicine Inha University, Incheon, Korea
혈중 아연 농도는 정상이나 모발 검사에서는 감소된 아연 농도를 보인 일과성 장병성 선단 피부염 1례
오경일, 김정희, 이지은, 임대현, 손병관
인하대학교 의과대학 소아과학교실
Correspondence: 
Dae Hyun Lim, Email: dhyunlim@inha.ac.kr
Abstract
Acrodermatitis enteropathica (AE) is a rare autosomal recessive disorder of early infancy, and is characterized by periorificial dermatitis, alopecia, and intractable diarrhea. Serum zinc levels are usually low in untreated patients and the oral administration of zinc sulfate can clear skin lesions and other symptoms. Although premature and cow`s milk-fed infants are at particular risk of developing AE, there have been a few reports about AE in term and breast-fed infants. We report a case of transient AE in a 4-month-old breast-fed infant. This patient suffered from diarrhea and dermatitis for more than a month. Her skin lesions were erythematous, scaly, crusted, psoriasiform, eczematous, with an eruption at the chin, and a periorificial disposition with involvement of the flexural areas of lower extremities. Her serum zinc level was almost normal at 129 g/dL (reference range: 60-121 g/dL), but the zinc level in her hair was low: 8 mg percent (reference range: 10-21 mg percent). Skin biopsy findings were consistent with AE. Seven days after zinc supplementation, the skin lesions and diarrhea improved. The authors recommend that a clinical trial of zinc supplementation be considered in cases where there are suspicious of AE, even when the serum zinc level is normal.
Key Words: Acrodermatitis enteropathica , Zinc


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