Journal of the Korean Pediatric Society 1991;34(2):292-298.
Published online February 28, 1991.
A case of hyperimmunoglobulin E syndrome.
Myeong Cheol Shinn, Sung Dong Choi, Byung Gyu Suh, Jin Han Kang, Joon Sung Lee
Department of Pediatrics, Catholic University Medical College, Seoul, Korea
고면역글로불린 E 증후군 1례*
신명철, 최성동, 서병규, 강진한, 이준성
가톨릭의과대학 소아과학교실
Received: 14 August 1990   • Accepted: 12 October 1990
Abstract
The hyperimmunoglobulin E syndrome is a primary immunodeficiency disorder characterized by serious recurrent bacterial infections of the skin and respiratory system in early life, and markedly elevated serum IgE level. These recurrent infections are generally subcutaneous abscesses caused by Staphylococcus aureus. Also, additional characteristrics of this syndrome include coarse facial appearance, chronic pruritic dermatitis and eosinophilia. The baisic immunologic defect mechanism in hyperimmunoglobulin E syndrome is not still fully understood, only suggest that abnormalities of the humoral immune response to staphylococcus aureus contribute to the predisposition to recurrent staphylococcal infection. We have experienced a case of hyperimmunoglobulin E syndrome in a 10-year-old boy who had suffered form severe recurrent multiple skin infections and chronic pruritic dermatitis since 7 month of age. A brief review of the related literature is presented.
Key Words: Hyperimmunoglobulin E syndrome, Recurrent skin infections




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