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

혈중 아연 농도는 정상이나 모발 검사에서는 감소된 아연 농도를 보인 일과성 장병성 선단 피부염 1례

  • Oh, Kyung Il (Department of Pediatrics, College of Medicine Inha University) ;
  • Kim, Jung Hee (Department of Pediatrics, College of Medicine Inha University) ;
  • Lee, Ji Eun (Department of Pediatrics, College of Medicine Inha University) ;
  • Lim, Dae Hyun (Department of Pediatrics, College of Medicine Inha University) ;
  • Son, Byong Kwan (Department of Pediatrics, College of Medicine Inha University)
  • 오경일 (인하대학교 의과대학 소아과학교실) ;
  • 김정희 (인하대학교 의과대학 소아과학교실) ;
  • 이지은 (인하대학교 의과대학 소아과학교실) ;
  • 임대현 (인하대학교 의과대학 소아과학교실) ;
  • 손병관 (인하대학교 의과대학 소아과학교실)
  • Received : 2006.10.12
  • Accepted : 2006.09.28
  • Published : 2007.02.15

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 $129{\mu}g/dL$ (reference range: $60-121{\mu}g/dL$), 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.

장병성 선단 피부염은 위장관에서 아연 흡수가 제대로 되지 않아 발생하는 질환으로서 주로 이유기의 영 유아에 호발하고 상염색체 열성 유전하는 질환이다. 홍반, 인설, 가피, 건선양 피부와 습진의 특징적인 피부 병변이 개구부와 사지 말단 부위에 대칭적으로 나타나고, 만성 설사, 탈모증, 조갑 주위염, 그리고 성장 장애가 나타나는 드문 질환이다. 혈중 아연 농도가 대부분에서 떨어져 있지만 정상 혈중 농도에서도 말초 조직 내의 아연 농도가 떨어지면 증상이 나타난다. 다른 원인 질환이 없고 만삭으로 정상 분만한 모유 수유 영아에서 조직학적으로 일치하며, 혈중 아연 농도는 정상이지만 모발에서 아연 농도가 떨어진 장병성 선단 피부염 증례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

Keywords

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