PMID- 15181495
OWN - NLM
STAT- MEDLINE
DCOM- 20041203
LR  - 20150901
IS  - 1684-1182 (Print)
IS  - 1684-1182 (Linking)
VI  - 37
IP  - 2
DP  - 2004 Apr
TI  - The hyperimmunoglobulin E syndrome.
PG  - 121-3
AB  - 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.
FAU - Hsu, Chen-Tong
AU  - Hsu CT
AD  - Department of Pediatrics, Yung Ho Branch, Cardinal Tien Hospital, Taipei, Taiwan, 
      ROC.
FAU - Lin, Yu-Tsan
AU  - Lin YT
FAU - Yang, Yao-Hsu
AU  - Yang YH
FAU - Chiang, Bor-Luen
AU  - Chiang BL
LA  - eng
PT  - Case Reports
PT  - Journal Article
PL  - England
TA  - J Microbiol Immunol Infect
JT  - Journal of microbiology, immunology, and infection = Wei mian yu gan ran za zhi
JID - 100956211
RN  - 37341-29-0 (Immunoglobulin E)
SB  - IM
MH  - Chemotaxis, Leukocyte
MH  - Child
MH  - Drug Resistance, Bacterial
MH  - Eosinophilia
MH  - Face
MH  - *Furunculosis/drug therapy/microbiology/surgery
MH  - Humans
MH  - Immunoglobulin E/blood
MH  - *Job Syndrome/blood/diagnosis/immunology
MH  - Leukocytosis
MH  - Lymphocyte Activation
MH  - Male
MH  - Methicillin Resistance
MH  - Neck/pathology
MH  - Scalp/pathology
MH  - Staphylococcus aureus/*drug effects/isolation & purification
EDAT- 2004/06/08 05:00
MHDA- 2004/12/16 09:00
CRDT- 2004/06/08 05:00
PHST- 2004/06/08 05:00 [pubmed]
PHST- 2004/12/16 09:00 [medline]
PHST- 2004/06/08 05:00 [entrez]
PST - ppublish
SO  - J Microbiol Immunol Infect. 2004 Apr;37(2):121-3.