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Hyperimmunoglobulinemia E Syndrome-A Case Report
Tsung-Jen Huang , Jia-Tsun Yen , Bor-Jeng Tsai
Dermatol Sinica 22 : 254-259, 2004

The hyperimmunoglobulinemia E syndrome (HIE syndrome) is a rare autosomal dominant primary immunodeficiency disorder. It is characterized clinically by hyperimmunoglobulinemia E, eosinophilia, chronic eczematoid dermatitis, recurrent bacterial infections particularly of the skin and the respiratory tract, facial and skeletal abnormality features. We report a 22-year-old female who suffered from HIE syndrome since early childhood. Her serum IgE was extremely elevated to 22,000 IU/ml. In addition to displaying the major features of HIE syndrome, she also developed cold abscesses and hyperextensible joints. Bacterial cultures taken from the cold abscess and the neck pustules all grew Staphylococcus aureus. Result of the skin biopsy showed spongiosis, superficial perivascular and interstitial mixed infiltrate of lymphocytes, histiocytes and some eosinophils. After the skin condition improved, we prescribed prophylactic antibiotics for her which greatly reduced the infections of the skin and the respiratory tract.

   
   
 
 
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