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Hyper-IgE syndrome. Presentation of three cases
F de la Torre Morín1, J C García Robaina, C Bonnet Moreno
1Allergy & Immunology Service, Ntra. Sra. de la Candelaria Hospital, Tenerife.
Allergologia Et Immunopathologia
|January 1, 1997
Summary
This study describes three children with Hyper-IgE syndrome, a condition marked by recurrent infections and high immunoglobulin E (IgE) levels. Treatment involved intravenous immunoglobulin, antibiotics, and ascorbic acid.
Area of Science:
- Immunology
- Pediatrics
- Genetics
Background:
- Hyper-IgE syndrome (HIES) is a primary immunodeficiency characterized by recurrent sinopulmonary and skin infections, eczema, and extremely elevated serum IgE levels.
- Historically, HIES has been described with varying clinical presentations, including recurrent staphylococcal abscesses and dermatitis.
- Despite advancements, the precise pathophysiology of HIES remains incompletely understood, highlighting the need for further research.
Purpose of the Study:
- To present three pediatric cases of Hyper-IgE syndrome with detailed clinical and immunological findings.
- To discuss the potential physiopathology and immunological alterations associated with HIES.
- To evaluate the therapeutic response to intravenous gammaglobulin, antibiotics, and ascorbic acid.
Main Methods:
- Case study of three patients diagnosed with Hyper-IgE syndrome.
- Clinical evaluation including recurrent infections, dermatitis, and abscesses.
- Laboratory investigations including IgE levels, eosinophilia, and neutrophil function tests (mitogen and chemotaxis responses).
Main Results:
- Patients presented with recurrent pulmonary infections, staphylococcal cold abscesses, and chronic dermatitis from infancy.
- Elevated IgE levels (>2,000 u/ml) and eosinophilia (>0.6 x 10(9) cells/l) were observed in all cases.
- Deficits in mitogen and chemotaxis responses were noted, indicating impaired immune function.
Conclusions:
- Hyper-IgE syndrome presents with a distinct triad of recurrent infections, elevated IgE, and immune dysregulation.
- The presented cases underscore the importance of early diagnosis and comprehensive immunological assessment in HIES.
- Treatment with intravenous gammaglobulin, antibiotics, and ascorbic acid showed potential benefits in managing HIES symptoms.