J Korean Pediatr Soc.  2001 Nov;44(11):1301-1304.

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

• Choi YJ
• Lee DI
• Kim HB

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.

Keyword

HyperIgE syndrome; Intravenous immunoglobulin

MeSH Terms

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
Anti-Bacterial Agents
Immunoglobulin E
Immunoglobulins
Immunoglobulins, Intravenous