Severe Periodontitis in a 5 year old Girl with Hyperimmunoglublin E Syndrome

Resident: Roberts

Date: 6/11/10

Article title: Severe Periodontitis in a 5 year old Girl with Hyperimmunoglublin E Syndrome

Author: Tsang, P et al.

Journal: Pediatric Dentistry

Volume: 27:1 pages: 73

Year: 2005

Type of article: Case report

Discussion:

Hyperimmunoglobulin E syndrome is a multisystem(HIES) disorder that affects the 1. dentition 2. skeleton 3. connective tissues 4. immune system. This disorder is also known as Jobs syndrome and Buckley’s syndrome. It can be autosomal dominant or recessive or may not have any apparent genetic link at all. Major manifestations of the disease include elevated serum E levels, chronic eczematoid dermatitis, recurrent skin abcesses, prominant forehead, deep set eyes, broad nasal bridge, mild prognathism, scoliosis, joint hyperextensibility and decreased bone density. Retention of primary teeth is a feature found in Autosomal dominant patients. In the recessive form skeletal and dental abnormalities are not usually present.

Case report

A 5 year old girl who has suspected autosomal recessive HIES syndrome presented with profuse bleeding, painful gingiva and generalized aggressive periodontitis. A microbiological examination detected P. gingivalis, T. forsythia, P, nigrescens, T. denticola, E. corrodens, and C. rectus but did not show the presence of A. A. All teeth except E, F, G showed 5 mm or more of probing depths and class 3 mobility. Advanced alveolar bone loss was present and only minor interproximal lesions present. Treatment included extraction of all primary teeth which resulted in elimination of soft tissue inflammation. Main management strategies are 1. Prophylatic antibiotics, 2. timely treatment of infections, 3. surgical intervention as necessary.