• Tuberculosis and Respiratory Diseases
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• v.52 no.6
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• pp.651-656
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• 2002

Hyperimmunoglobulin E syndrome, otherwise known as Job's syndrome, is an immune disorder characterized by an abnormal elevation of the circulating immunoglobulin E level, and recurrent infections of the skin and sinopulmonary tract. The syndrome has various pulmonary features, e.g., pneumonia, pneumatocele, pneumothorax, lung abscesses and empyema. We report a case of hyperimmunoglobulin E syndrome, with various respiratory clinical manifestation. Medical therapy, including prophylactic antibiotics, has been the cornerstone for the treatment of hyperimmunoglobulin E syndrome. Even if surgical intervention is required, minimal pulmonary parenchymal resection is recommended.

• Advances in pediatric surgery
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• v.2 no.2
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• pp.151-155
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• 1996

Hyperimmunoglobulin E syndrome is a relatively rare primary immunodeficiency syndrome characterized by recurrent infection, abscess formation and marked elevation of serum IgE level. The common infectious organism is Staphylococcus aureus and recurrent infection indicates some defects in the immunologic system. Although the infection can affect various organs, gastrointestinal tract involvement is rare and only one case of colon perforation has been previously reproted. Herein we report another one case of colon perforation which ocurred in an 8-year-old girl with hyper immunoglobulin E syndrome. The patient was admitted to the hospital due to an abscess on right neck. The diagnosis of hyper immunoglobulin E syndrome was made because she had eczematoid dermatitis on the face, pneumatocele on left upper lung field and markedly elevated serum IgE level(>15,000 IU/ml) with a past histories of frequent scalp abscesses and otitis media. Abdominal pain developed on the 13th day of admission and abdominal plain X-ray revealed free air. An exploratory laparatomy was performed and two free perforations of the transverse colon were noted. Segmental resection and double barrel colostomy were performed. Colostomy closure was done 4 month later and she had no gastrointestinal problem during a follow up period of 15 months.

• Pediatric Infection and Vaccine
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• v.10 no.1
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• pp.127-131
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• 2003

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.

• Korean journal of dermatology
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• v.56 no.9
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• pp.573-574
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• 2018

• Clinical and Experimental Pediatrics
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• v.53 no.4
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• pp.592-597
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• 2010

Hyperimmunoglobulin E syndrome (HIES) is a rare immunodeficiency disease which is characterized by high serum IgE levels, eczema, and recurrent infections. Herein we present the case of a patient with HIES associated with STAT3 gene ($stat3$) mutation. A 16 year-old girl was admitted to our hospital due to hemoptysis caused by pneumonia with bronchiectasis. She had a history of recurrent skin and respiratory tract infections, such as pneumonia caused by MRSA (methicillin-resistant $Staphylococcus$ $aureus$) and $Pseudomonas$ $aeruginosa$. On physical examination, a broad round shaped nose, oral thrush, and chronic eczematous skin rash over her whole body were found. Laboratory data showed an elevated eosinophil count ($750/{\mu}L$) and total IgE level (5,001 U/mL). The patient's National Institutes of Health (NIH) score for HIES was 44. Direct sequencing of the STAT3 gene revealed that the patient was heterozygous for a missense mutation in the DNA binding domain of the STAT3 protein (c.1144C>T, p. Arg382Trp). HIES should be suspected in patients with recurrent infections and can be confirmed by clinical scoring and genetic analysis.