Pediatrics & Therapeutics
Open Access

Abstract

Primary Teeth Retention as the Sole Presentation of Hyper IgE Syndrome: A Case Report

Sepideh Darougar*, Atena Ramezanali, Mohammad Gharagozlou, Zahra Chavoshzadeh and Marzieh Heidarzadeh Arani

Hyper IgE syndrome is a rare primary immunodeficiency disorder characterized by cold staphylococcal abscesses, recurrent respiratory infections, skeletal and coarse facial features and dentition abnormalities associated with high serum IgE levels. However, some cases remain asymptomatic or present with few or atypical manifestations of the disease. This is a report of a 14-year-old boy presented with delayed teeth eruption and coarse facies in addition to several asthma attacks. Further investigations revealed elevated serum IgE levels. Hyper IgE syndrome was considered due to recurrent sinus infections and retention of his primary teeth which was later confirmed by mutation analysis.

Published Date: 2024-06-12; Received Date: 2020-06-08