A Case of the Use of Intravenous Immunoglobulin of Treatment for HyperIgE Syndrome.
- Author:
Yoon Ji CHOI
1
;
Dong IL LEE
;
Hong Bae KIM
Author Information
1. Department of Pediatrics, Wallace Memorial Baptist Hospital, Pusan, Korea.
- Publication Type:Case Report
- Keywords:
HyperIgE syndrome;
Intravenous immunoglobulin
- MeSH:
Abscess;
Anti-Bacterial Agents;
Deficiency Diseases;
Diagnosis;
Ear Canal;
Eczema;
Hospitalization;
Humans;
Immunization, Passive;
Immunoglobulin E;
Immunoglobulins*;
Immunoglobulins, Intravenous;
Infant;
Infant, Newborn;
Job Syndrome;
Lung Diseases;
Penetrance;
Scalp
- From:Journal of the Korean Pediatric Society
2001;44(11):1301-1304
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
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.
