Gingival enlargement as a manifestation of tuberous sclerosis: case report and periodontal management.

BACKGROUND: Tuberous sclerosis is an autosomal-dominant inherited disease involving many organs of the body. Oral manifestations include gingival enlargement, fibromas, and dental enamel pitting. The report presents a case of tuberous sclerosis with gingival enlargement histologically consistent with angiofibroma, describes its successful periodontal management, and reviews the literature associated with oral manifestations of tuberous sclerosis. METHODS: A 26-year-old white male presented to the Department of Periodontics and Implant Dentistry, New York University College of Dentistry, with a diagnosis of tuberous sclerosis and a chief complaint of gingival enlargement affecting mastication and esthetics. Following a complete medical history review, consultation with the patient's medical team at New York University Medical Center, and a thorough oral and periodontal examination, a treatment plan was developed that included oral hygiene instructions, mechanical debridement, and periodontal reevaluation. This was followed by gingivectomy, which provided improved function and esthetics. Excised tissue was submitted for histologic examination. The patient was followed every 2 months for assessment of the outcome of the surgical treatment. An extensive search of the dental and dermatologic literature was performed on MEDLINE. RESULTS: Histologic examination of the gingival tissue revealed features consistent with angiofibroma. Fifteen months following gingivectomy, the contours and gingival surface appearance remained normal. CONCLUSIONS: The gingival enlargement was histologically consistent with the characteristic angiofibromas of tuberous sclerosis. The gingival enlargement responded very well to gingivectomy and periodontal maintenance.

Papillon-Lefèvre syndrome: a successful outcome.

BACKGROUND: Papillon-Lefèvre syndrome (PLS) is a rare autosomal recessive condition manifested clinically by hyperkeratosis of the palms and soles and rapidly progressive periodontitis resulting in loss of deciduous and permanent teeth. This case report describes the clinical periodontal findings and treatment of a 10-year-old male patient with PLS. The patient provided informed consent, and the study was conducted in accordance with the Helsinki Declaration of 1975, as revised in 2000. METHODS: Upon initial presentation, a full periodontal examination was completed. Conventional probing depths, clinical attachment levels (CAL), gingival index (GI), and plaque index (PI) were measured prior to initial therapy, which involved oral hygiene instruction and scaling and root planing. At reevaluation, initial treatment proved unsuccessful, and a surgical approach with concomitant systemic antibiotic therapy was implemented. In addition, the patient's dermatologist treated his palmoplantar keratoderma with systemic retinoids. Subsequently, the patient was placed on a strict 3-month maintenance protocol and was evaluated over a period of 1 year. RESULTS: Initial treatment with mechanical therapy, oral hygiene instruction, frequent recalls, and systemic antibiotics did not yield efficacious results. However, with the addition of surgical treatment, a favorable clinical outcome was obtained. CONCLUSIONS: Numerous treatment regimens for the periodontal disease seen in PLS can be found in the literature. We demonstrate successful treatment of the periodontal disease seen in this condition using mechanical therapy, systemic antibiotics, and surgical modalities; over a period of 1 year, we were able to achieve significant reductions in gingival inflammation and erythema.