The hyperimmunoglobulin E syndrome

Chen-Tong Hsu^1,2, Yu-Tsan Lin², Yao-Hsu Yang², Bor-Luen Chiang^2
1Department of Pediatrics, Yung Ho Branch, Cardinal Tien Hospital, Taipei; and ²Department 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:

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.