Korean J Pediatr Infect Dis.  2003 May;10(1):127-131. 10.14776/kjpid.2003.10.1.127.

A Case of Hyper IgE Syndrome with Severe Ulcer and Granuloma at the Site of BCG Inoculation

• Rho JA
• Rho YI
• Moon KR
• Park YB
• Yang ES

• KMID: 2440108
• DOI: http://doi.org/10.14776/kjpid.2003.10.1.127

Abstract

Hyperimmunoglobulin E syndrome(HIES) is a primary immunodeficiency characterized by severe recurrent soft-tissue infections, pneumonias, pruritic dermatitis and markedly elevated serum IgE levels. Pneumatocele and empyema develop as result of recurrent pneumonias. It is inherited in an autosomal dominant fashion, with variable expressivity. We experienced the case of a 10 month old girl with hyper IgE syndrome. Patient was admitted for local ulceration and muscle soreness at the site of BCG inoculation. The diagnosis of hyper IgE syndrome was made because she had a coarse facial appearance, pruritic dermatitis, pneumatocele and markedly elevated serum IgE level with a past history of frequent skin abscess and pneumonia

Keyword

Hyperimmunoglobulin E syndrome; BCG

MeSH Terms

Abscess
Dermatitis
Diagnosis
Empyema
Female
Granuloma*
Humans
Immunoglobulin E
Job Syndrome*
Myalgia
Mycobacterium bovis*
Pneumonia
Skin
Ulcer*
Immunoglobulin E