Related Experiment Video
Updated: Aug 9, 2026
10:27
Recognition of Epidermal Transglutaminase by IgA and Tissue Transglutaminase 2 Antibodies in a Rare Case of Rhesus Dermatitis
Published on: December 15, 2011
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.
Related Concept Videos
01:28Peptic Ulcer Disease III: Clinical Manifestations and Diagnostic Studies
Peptic ulcer disease (PUD) presents with diverse symptoms depending on the location and severity of the ulcer. Clinical manifestations of peptic ulcer include dull pain and a burning sensation in the mid-epigastric region.
Few clinical manifestations differentiate gastric ulcers from duodenal ulcers. Distinctions in the location, timing, and pain relief are crucial for healthcare providers in differentiating between gastric and duodenal ulcers during clinical assessments.
Few clinical manifestations differentiate gastric ulcers from duodenal ulcers. Distinctions in the location, timing, and pain relief are crucial for healthcare providers in differentiating between gastric and duodenal ulcers during clinical assessments.
01:30Irritable Bowel Syndrome II: Clinical Features and Diagnostic Evaluation
Irritable Bowel Syndrome II: Clinical Features and Diagnostic Evaluation
Irritable Bowel Syndrome (IBS) is classified into subtypes based on the predominant bowel habits as determined by the Bristol Stool Form Scale (BSFS). The subtypes are:
Irritable Bowel Syndrome (IBS) is classified into subtypes based on the predominant bowel habits as determined by the Bristol Stool Form Scale (BSFS). The subtypes are:
01:19Type I Diabetes III: Clinical Manifestations
Type 1 diabetes mellitus typically presents with rapid-onset symptoms due to the body’s inability to utilize glucose in the absence of insulin. Since insulin is required for glucose uptake into cells, its deficiency leads to hyperglycemia and cellular energy deprivation, resulting in characteristic clinical features.Polyuria and PolydipsiaOne of the earliest, most prominent symptoms is polyuria (excessive urination). When blood glucose concentrations rise above the renal threshold, the kidneys...
01:29Hyperglycemia
Hyperglycemia is an abnormally high blood glucose level. It is diagnosed by fasting glucose ≥126 mg/dL, 2-hour oral glucose tolerance test (or OGTT) ≥200 mg/dL, random glucose ≥200 mg/dL with symptoms, or HbA1c ≥6.5%. However, HbA1c results may be unreliable in certain conditions, such as anemia or hemoglobinopathies, and the diagnosis should be confirmed unless classic symptoms are present. Postprandial hyperglycemia is typically considered significant when glucose levels exceed 180 mg/dL two...
01:26Gastritis II: Pathophysiology
The pathophysiology of gastritis begins with the colonization of the stomach lining by Helicobacter pylori (H. pylori). This bacterium spreads mainly via the oral-oral route through saliva or shared utensils, and can also be transmitted in overcrowded or unhygienic environments through contaminated water, despite its brief survival outside the body.ColonizationOnce ingested, H. pylori enters the stomach and begins colonization by navigating through the mucus layer lining the stomach wall. It...
01:20Inflammatory Bowel Disease IV: Clinical Manifestations
Inflammatory bowel disease (IBD) encompasses two major chronic disorders—ulcerative colitis and Crohn’s disease—each characterized by relapsing episodes of gastrointestinal inflammation. Although they share certain clinical features, their patterns of involvement and manifestations differ in ways that aid diagnosis and guide management.Ulcerative ColitisUlcerative colitis is limited to the colon and rectum and involves continuous inflammation of the mucosal layer. The disease course is marked...