Dental manifestations of a pediatric patient with hyperimmunoglobulin e syndrome: a case report

A 7-year-old girl with hyperimmunoglobulin E syndrome (HIES) presented to our clinic with tooth agenesis in both primary and permanent dentitions. The patient's serum immunoglobulin E level was elevated at 17,091 IU/ml, and her medical history indicated the occurrence of HIES, numerous skin abscesses, and recurrent infection by bacteria and/or fungi such as Candida from birth. She also suffered from heart disease. Dental manifestations included extensive caries, impaired root resorption of primary mandibular central incisors and absence of primary mandibular canines and permanent mandibular lateral incisors. Intraoral phenotypes in HIES patients have already been reported in detail, but no previous report has described abnormalities in the number of primary teeth in HIES patients. The purpose of this report was to describe the dental manifestations in the primary dentition of a hyperimmunoglobulin E syndrome patient and to emphasize the importance of preventive dental management from early childhood in compromised children, such as those with HIES.