All issues > Volume 44(11); 2001

Case Report

J Korean Pediatr Soc. 2001;44(11):1301-1304. Published online November 15, 2001.

A Case of the Use of Intravenous Immunoglobulin of Treatment for HyperIgE Syndrome

Yoon Ji YJ Choi¹, Dong IL DI Lee¹, Hong Bae HB Kim¹

¹Department of Pediatrics, Wallace Memorial Baptist Hospital, Pusan, Korea

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.

Keywords :HyperIgE syndrome, Intravenous immunoglobulin