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題名 | Hyperimmunoglobulinemia E Syndrome--A Case Report=免疫球蛋白E過高徵候群--一病例報告 |
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作者姓名(中文) | 黃宗仁; 顏嘉村; 蔡博正; | 書刊名 | 中華皮膚科醫學雜誌 |
卷期 | 22:3 2004.09[民93.09] |
頁次 | 頁254-259 |
分類號 | 415.69 |
關鍵詞 | 免疫球蛋白E過高徵候群; 自體顯性遺傳免疫缺乏症; Hyperimmunoglobulinemia E syndrome; Hyper-IgE syndrome; Job's syndrome; IgE; Staphylococcus aureus; |
語文 | 英文(English) |
中文摘要 | 免疫球蛋白過多徵候群是一種罕見的自體顯性遺傳免疫缺乏症,臨床表現為高血E型免疫球蛋白症、嗜伊紅球增多、慢性濕疹樣皮膚炎、反覆性的皮膚和呼吸道細菌感染,此外有病患會有獨特的面容、骨骼異常等。我們在此報告一22歲女性血清E型免疫球蛋白高達 22,000 IU/ml,她除了表現出E型免疫球蛋白過多徵候群的主要症狀以外,皮下有冷膿瘍、關節有過度伸張等特徵。從皮膚膿疱和皮下冷膿瘍中都培養出金黃色葡萄球菌。皮膚切片顯示上皮細胞海綿化,在表層血管周圍和間質內有著淋巴球、組織球和嗜伊紅球浸潤。在皮膚狀況控制以後,我們給予預防性的抗生素治療。之後病人的皮膚感染和呼吸道感染已大幅減少。 |
英文摘要 | 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 Stayphlococcus aureos. Resuslt 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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