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Volume 37, Number 2, April 2004

The hyperimmunoglobulin E syndrome


Chen-Tong Hsu, Yu-Tsan Lin, Yao-Hsu Yang, Bor-Luen Chiang
Department of Pediatrics, Yung Ho Branch, Cardinal Tien Hospital, Taipei; and Department of Pediatrics, National Taiwan University Hospital, Taipei, Taiwan, ROC

Received: July 15, 2003    Revised: August 12, 2003    Accepted: August 28, 2003   

 

Corresponding author:

Dr. Bor-Luen Chiang, Department of Pediatrics, National Taiwan University Hospital, No. 7, Chung-Shan South Road, Taipei, Taiwan 110, ROC. E-mail: gicmbor@ha.mc.ntu.edu.tw This e-mail address is being protected from spam bots, you need JavaScript enabled to view it

 



 

Methods:

Hyperimmunoglobulin E syndrome is a primary immunodeficiency disease characterized by markedly high titers of serum immunoglobulin E (IgE), chronic eczema, recurrent staphylococcal infections, pneumatoceles, reduced neutrophil chemotaxis, and variable impaired T cell function. There are no clinical tools for diagnosis and definitive laboratory investigation. Variability of presentation makes it easy to confuse the diagnosis with that of severe atopy or other rare immunodeficiencies. We report a case of a 6-year-old boy with hyperimmunoglobulin E syndrome with recurrent methicillin-resistant Staphylococcus aureus furunculosis. Physical examination revealed a peculiar facial appearance, pruritic dermatitis, and furunculosis over the scalp, neck, and back. Laboratory investigation revealed mild leukocytosis with eosinophilia, a very high immunoglobulin E level, defective neutrophil chemotaxis, and impaired lymphocyte proliferation to anti-CD3/CD28 monoclonal antibodies. The boy was discharged without incident after 2 weeks of antibiotic therapy and debridement.



 

Key words:

Furunculosis, hyper-IgE syndrome, methicillin resistance, Staphylococcus aureus



 



 

J Microbiol Immunol Infect 2004;37:121-123.