RARE PRIMARY IMMUNODEFICIENCY – HYPER-IgE-SYNDROME: CASE REPORT AND LITERATURE REVIEW

Abstract

The article is dedicated to one of the rare primary immunodeficiency pathologies – hyper-IgE-syndrome. The authors present a clinical case from their own practice and a literature review on this disease. Inheritance, pathogenesis, clinical manifestations and hyper-IgE-syndrome diagnostics issues are also examined in the article. The article shows differences of disease course at different inheritance types – autosomal-dominant and autosomal-recessive.

Keywords

• primary immunodeficiency
• yper-ige-syndrome
• inheritance
• etiology
• pathogenesis
• clinical course
• diagnostics
• treatment
• children