Hyper-IgE Syndrome

Synonyms

4

Overview

Hyper-IgE syndrome (HIES, also called Job-Buckley syndrome, Job's syndrome and Buckley syndrome), is a heterogeneous group of disorders characterized by recurrent staphylococcal infections, unusual eczema-like skin rashes, severe lung infections that result in pneumatoceles (balloon-like lesions that may be filled with air or pus or scar tissue) and very high concentrations of the serum antibody IgE.

Symptoms

• Staphylococcus aureus infections 
• Recurrent abscesses 
• Skin abscesses 
• Lung abscesses 
• Recurrent pneumonia

Causes

In autosomal dominant-HIES an heterozygous mutation in the gene encoding the transcription factor STAT3 was found. Mutations and deletions in the DOCK8 gene were found with autosomal recessive-HIES.

Prevention

Not knowen, good general hygiene can help.

Diagnosis

The signs and symptom information on this page attempts to provide a list of some possible signs and symptoms of hyper-IgE syndrome. This medical information about signs and symptoms for hyper-IgE syndrome has been gathered from various sources, may not be fully accurate, and may not be the full list of hyper-IgE syndrome signs or hyper-IgE syndrome symptoms. Furthermore, signs and symptoms of hyper-IgE syndrome may vary on an individual basis for each patient. Only your doctor can provide adequate diagnosis of any signs or symptoms and whether they are indeed hyper-IgE syndrome symptoms.

Prognosis

Hyper-IgE syndrome is a chronic life condition, every new infection needs treatment.

Treatment

Most patients with hyper-IgE syndrome are treated with chronic antibiotics to help protect them from staphylococcal infections. Good skin care is also important in patients with hyper-IgE syndrome. High-dose intravenous gamma-globulin has also been suggested for the treatment of severe eczema in patients with HIES and atopic dermatitis.