Pediatric Infection and Vaccine

The Korean Society of Pediatric Infectious Diseases (대한소아감염학회)
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A Case of Hyper IgE Syndrome with Severe Ulcer and Granuloma at the Site of BCG Inoculation

비씨지 접종부위에 심한 궤양과 육아종을 보인 고면역글로불린 E 증후군 1례

  • 노정아 (조선대학교 의과대학 소아과학교실) ;
  • 노영일 (조선대학교 의과대학 소아과학교실) ;
  • 문경래 (조선대학교 의과대학 소아과학교실) ;
  • 박영봉 (조선대학교 의과대학 소아과학교실) ;
  • 양은석 (조선대학교 의과대학 소아과학교실)
  • Published : 2003.05.31
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Abstract

Hyperimmunoglobulin E syndrome(HIES) is a primary immunodeficiency characterized by severe recurrent soft-tissue infections, pneumonias, pruritic dermatitis and markedly elevated serum IgE levels. Pneumatocele and empyema develop as result of recurrent pneumonias. It is inherited in an autosomal dominant fashion, with variable expressivity. We experienced the case of a 10 month old girl with hyper IgE syndrome. Patient was admitted for local ulceration and muscle soreness at the site of BCG inoculation. The diagnosis of hyper IgE syndrome was made because she had a coarse facial appearance, pruritic dermatitis, pneumatocele and markedly elevated serum IgE level with a past history of frequent skin abscess and pneumonia.

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