Hyper IgE Syndrome: a rare cause of bronchiectasis

Introduction: Job's syndrome (Hyper IgE Syndrome – HIES) is a primary immunodeficiency characterized by raised levels of IgE, eczema and recurrent skin and lung infections in childhood, affecting fewer than 1 per million patients. Although defect in STAT3 protein plays a role, diagnosis is based on clinical and laboratory features. Therapy includes antibiotics, with Staph. Aureus being the most common pathogen. Pneumatoceles and bronchiectasis formed during the healing process predispose to Gram (-) and fungal infections. Finally, HIES is also associated with an increased risk of malignancy. Aim: The presentatio…