Síndrome de Hiper IgE: relato de caso

Abstract

Objective: To report a case of Hyper Immunoglobulin E Syndrome treated at a university hospital. This syndrome is very rare and its clinical presentation is often mistaken for other more frequent diseases, delaying diagnosis and initiation of treatment. Also discussed is how to make the selection and investigation of suspected cases and treatment. Description: CBS, female, now 9 years and 11 months old, was brought to our clinic with a history of recurrent respiratory infections, severe atopic eczema, multiple food allergies, difficulty ingesting solid and malnutrition. Numerous suspected diagnoses were thought to it until considering the possibility of a primary immunodeficiency. Comment: It is important to make suspicion of immunodeficiency in all cases of skin rash during the first months of life, especially when associated with recurrent infections, with or without a family history. Early diagnosis and introduction of antibiotic prophylaxis should be made to prevent worsening of the skin and the possibility of life- threatening infectious complications.