title: Hyper-IgE syndrome: dental implications.
abstract: Hyper-IgE syndrome (HIES) is a rare multisystem disorder with both immunologic and nonimmunological features. It is characterized by extremely elevated IgE serum levels, eczema, and recurrent skin and pulmonary infections. Dental anomalies are often included, such as retention of deciduous teeth together with ectopic eruption or noneruption of permanent teeth. Severe susceptibility to caries and mycotic infections, insufficient transversal diameter of the palate, mucosal plaques, and fissures typically located on the tongue and on the palate are often present. The aim of this study was to review the literature and to report a 6-year observation of 6 patients with HIES (aged 8-39 years) with focus on their oral problems and the treatment provided. The importance of the role of the dentist both in early diagnosis of this syndrome and in monitoring oral conditions was stressed. The dentist can prevent infective complications and intercept the development of malocclusion with a reduction of the need for complex treatment.
authors: Department of Orthodontics, IRCCS Cà Granda - Ospedale Maggiore Policlinico, University of Milan, Milan, Italy.
authors: Luca
authors: L
authors: Esposito
authors: Laura
authors: L
authors: Poletti
authors: Cinzia
authors: C
authors: Maspero
authors: Alessandro
authors: A
authors: Porro
authors: Maria Cristina
authors: MC
authors: Pietrogrande
authors: Paola
authors: P
authors: Pavesi
authors: Rosa Maria
authors: RM
authors: Dellepiane
authors: Giampietro
authors: G
authors: Farronato
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D003733:Dental Caries Susceptibility; D004802:Eosinophilia / Q000209:etiology; D019066:Facies; D006801:Humans; D007589:Job Syndrome / Q000150:complications* / Q000235:genetics* / Q000276:immunology / Q000473:pathology; D009154:Mutation; D021362:Palate, Hard / Q000473:pathology; D012141:Respiratory Tract Infections / Q000209:etiology; D050796:STAT3 Transcription Factor / Q000235:genetics; D014063:Tongue, Fissured / Q000209:etiology; D014071:Tooth Abnormalities / Q000209:etiology; D014079:Tooth Eruption, Ectopic / Q000209:etiology*; D014094:Tooth, Deciduous / Q000503:physiopathology*; D055815:Young Adult
chemical_list: D050796:STAT3 Transcription Factor