All issues > Volume 34(2); 1991

Original Article

J Korean Pediatr Soc. 1991;34(2):292-298. Published online February 28, 1991.

A case of hyperimmunoglobulin E syndrome.

Myeong Cheol Shinn¹, Sung Dong Choi¹, Byung Gyu Suh¹, Jin Han Kang¹, Joon Sung Lee¹

¹Department of Pediatrics, Catholic University Medical College, Seoul, Korea

Received: August 14, 1990;  Accepted: October 12, 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.

Keywords :Hyperimmunoglobulin E syndrome;Recurrent skin infections