title: Severe periodontitis in a 5-year-old girl with hyperimmunoglobulin E syndrome.
abstract: The hyperimmunoglobulin E syndrome (HIES) is a multisystem disorder that affects the: (1) dentition; (2) skeleton; (3) connective tissues; and (4) immune system. Little is known about periodontal manifestations of the syndrome. The purpose of this report was to describe a 5-year-old girl with suspected autosomal-recessive HIES syndrome who revealed profusely bleeding and painful gingiva and generalized aggressive periodontitis. A polymerase chain reaction (PCR)-based microbiological examination detected Porphyromonas gingivalis, Tannerella forsythia, Prevotella nigrescens, Treponema denticola, Eikenella corrodens, and Campylobacter rectus in the deep periodontitis lesions. The extraction of all deciduous teeth due to a poor prognosis and risk of systemic infection led to resolution of the oral inflammation. Long-term follow-up is required to determine the periodontal prognosis of the erupting permanent teeth.
authors.affiliation: Department of Dentistry, British Columbia's Children's Hospital, Vancouver, British Columbia, Canada. phoebetis@shaw.ca
authors.forename: P
authors.initials: P
authors.lastname: Tsang
authors.forename: G
authors.initials: G
authors.lastname: Derkson
authors.forename: R
authors.initials: R
authors.lastname: Priddy
authors.forename: A K
authors.initials: AK
authors.lastname: Junker
authors.forename: J
authors.initials: J
authors.lastname: Slots
authors.forename: H
authors.initials: H
authors.lastname: Larjava
mesh_terms: D001421:Bacteria, Anaerobic / Q000302:isolation & purification; D002675:Child, Preschool; D003241:Consanguinity; D005260:Female; D019214:Gingival Overgrowth / Q000209:etiology; D006801:Humans; D007589:Job Syndrome / Q000150:complications*; D010518:Periodontitis / Q000209:etiology* / Q000382:microbiology; D014081:Tooth Extraction; D014094:Tooth, Deciduous / Q000601:surgery