https://scholars.lib.ntu.edu.tw/handle/123456789/568017
標題: | The hyperimmunoglobulin E syndrome | 作者: | Hsu C.-T. YU-TSAN LIN YAO-HSU YANG BOR-LUEN CHIANG |
公開日期: | 2004 | 卷: | 37 | 期: | 2 | 起(迄)頁: | 121-123 | 來源出版物: | Journal of Microbiology, Immunology and Infection | 摘要: | 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. |
URI: | https://www.scopus.com/inward/record.uri?eid=2-s2.0-3042580562&partnerID=40&md5=b10ce7ad9eb0b738775dc2cc4332c89c https://scholars.lib.ntu.edu.tw/handle/123456789/568017 |
ISSN: | 1684-1182 | SDG/關鍵字: | hyperimmune globulin; immunoglobulin E; monoclonal antibody CD3; antibiotic therapy; article; back; case report; debridement; dermatitis; eczema; eosinophilia; facies; furunculosis; human; hyperimmunoglobulin E syndrome; immune deficiency; immunoglobulin blood level; laboratory test; leukocytosis; lymphocyte proliferation; male; methicillin resistant Staphylococcus aureus; neck; neutrophil chemotaxis; physical examination; pneumatocele; preschool child; recurrent infection; scalp; skin disease; Staphylococcus infection; Chemotaxis, Leukocyte; Child; Drug Resistance, Bacterial; Eosinophilia; Face; Furunculosis; Humans; Immunoglobulin E; Job's Syndrome; Leukocytosis; Lymphocyte Activation; Male; Methicillin Resistance; Neck; Scalp; Staphylococcus aureus |
顯示於: | 醫學系 |
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