| A Case of the Use of Intravenous Immunoglobulin of
Treatment for HyperIgE Syndrome |
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Yoon Ji Choi, Dong IL Lee, Hong Bae Kim |
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Department of Pediatrics, Wallace Memorial Baptist Hospital, Pusan, Korea |
| 고면역글로불린 E 증후군에서 Intravenous Immunoglobulin을 치료에 사용한 1례 |
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최윤지, 이동일, 김홍배 |
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왈레스기념 침례병원 소아과 |
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| Abstract |
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Hyper-IgE syndrome is a rare congenital immune deficiency disease characterized by severe eczema, recurrent infection of the sinopulmonary tract, cold subcutaneous abscess and high serum IgE levels. It is an autosomal dominant disease with incomplete penetrance. We examined the case of a 12 month old infant with hyperIgE syndrome since neonate. The diagnosis of hyper IgE syndrome was made because he had eczema on the face and scalp, subcutaneous absecesses on both external ear canals and markedly elevated serum IgE level with a past history of frequent pulmonary disease. Topical steroid and systemic antibiotics had been unhelpful in treating the eczema and the pulmonary disease. The patient received IVIG for severe eczema and the pulmonary infection. The eczema and pulmonary infection were significantly improved and the patient was discharged after 35days of hospitalization. After 10 months of immunoglobulin therapy, symptoms and general appearance were markedly improved. IVIG can be considered as a choice in the treatment of hyper IgE syndrome. |
| Key Words:
HyperIgE syndrome, Intravenous immunoglobulin |
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