: Severe Periodontitis in a 5 year old Girl with Hyperimmunoglobulin E Syndrome 4/23/2010

Department of Pediatric Dentistry
Lutheran Medical Center

Resident’s Name: Craig Elice Date: 04/23/2010
Article title: Severe Periodontitis in a 5 year old Girl with Hyperimmunoglobulin E Syndrome
Author(s): Tsang P, Derkson G, Et al
Journal: Pediatric Dentistry 27:1
Month, Year: 2005
Major topic: Severe periodontal disease in Hyperimmunoglobulin E Syndrome
Type of Article: Case Report
Purpose: This article describes a 5 year old female with probable Autosomal Recessive HgE Syndrome who had severe perio in her entire primary dentition.
Background: HIES a.k.a. Job’s syndrome or Buckley syndrome has an autosomal dominant and Autosomal Recessive form The AD form is recognized by an increased IgE, chonic eczema, recurrent skin abscesses, lung staph infections, and a typical facies of a prominent forehead, deep set eyes, broad nasal bridge, mild prognathism, scoliosis, joint hyperextensibility, and a decreased bone density leading to fractures. The AR form also has an increased susceptibility to severe fungal and viral infections and a higher incidence of vascular and infectious central nervous syndrome complications. A diagnosis is based on a NIH score. Management is based on prophylactic antibiotics, localdebridement and surgical incision and drainage. A limited number of studies showed delayed eruption of permanent teeth.
Discussion: The patient was the first born child of a Kurdish couple who were first cousins. In infancy, the child developed severe eczema which were complicated by recurrent staph aureus and Candida albicans supra infections. The patient also had recurrent otitis media, hospitalization 5x for pneumonia, 1x for Staph Aureus sepsis. At age 3, she developed severe HSV-1 gingivostomatitis, and shingles. Osteopenia led to 2 tibial fractures.Lastly she exhibited failure to thrive. Extraoral exam revealed dry cracked lips, eczema like dryness of cheeks. Intraoral exam showed a smooth tongue with deep fissures. Periodontal evaluation revealed red edematous rolled margins with bleeding on probing and pocket depths of up to 9mm on all primary teeth. The treatment plan involved extraction of all prmary teeth to prevent infection from developing as the secondary teeth erupted and to prevent development of pneumonia secondary to a dental infection. The author suggests that rapid perio disease is caused by the f=effects of highly virulent perio pathologic bacteria, deficient PMN leukocytes repnses, and an increase in potent bone resorbing cytokines and decrease in bone resorption inhibitory cytokines.
Conclusion: this is the first reported case of severe periodontal disease related to Hyper IgE syndrome. Other more typical features include recurrent skin and lung staph infections, chronic eczematoid dermatitis, and elevated serum IgE levels. Despite advances in our ability to diagnosis the disease, management remains the same with prophylactic antibiotic therapy, timely treatment of infections, and surgical inbervention as needed.
Assessment of article: Good article on a very rare condition.