Rabson-Mendenhall syndrome: two case reports and a brief review of the literature.

Rabson-Mendenhall syndrome is a rare, autosomal recessive disorder characterized by insulin resistance syndrome, growth retardation, coarse and senile-looking faces, mental precocity, early dentition, and pineal hyperplasia. Mutations of the insulin receptor gene affecting insulin action appear to be the basic mechanism underlying this syndrome. We report on Rabson-Mendenhall syndrome in two siblings and briefly review the literature.

Sturge-Weber syndrome: physician's dream; surgeon's enigma.

Sturge-Weber syndrome (SWS) is a neurocutaneous syndrome consisting of port wine stain (facial nevus flammeus), congenital glaucoma and underlying anomalous leptomeningeal venous plexus with lack of normal cortical venous drainage. Neuromuscular, ocular, dermatologic and oral manifestations are variously present with this syndrome. The syndrome presents with various oral findings and the diagnosis of these cases is based primarily on clinical findings. Considering the complications that may be encountered during the treatment and the patient's own perception of the condition, it is imperative that the diagnostician and surgeon have thorough knowledge of the condition. Here we report a case of SWS with facial angiomas, seizures and intracranial calcifications.

Klippel-Trénaunay syndrome (angio osteohypertrophy syndrome): a report of 3 cases.

Klippel-Trénaunay syndrome was first reported in 1900 by Klippel and Trénaunay. The syndrome is characterized by the triad of vascular nevi, venous varicosity, and hyperplasia of soft tissue-and possibly bone-in the affected area. The original description was made with regard to the extremities. The syndrome is diagnosed on the basis of 2 or 3 symptoms. The 3 patients reported here presented with hemangioma and hypertrophy of the jawbone and concomitant malocclusion. There were clinically no obvious varicose veins. It appears that gravity plays an important role in the venous drainage from the head and neck region, and this could be the reason for the rarity of varicose veins in the head and neck region when compared with lower extremities. In addition, 2 of the 3 cases showed exophytic pedunculated growth arising from the gingiva, which has not been reported in any previous case reports. The following report presents the clinical features in the orofacial region and highlights the clinical significance of this syndrome.

Cowden's syndrome: a case report.

Cowden's syndrome, a rare genodermatosis of autosomal-dominant inheritance with variable expressivity, is characterized by a combination of ectodermal, mesodermal, and endodermal hamartomas that may involve the skin, mucous membranes, breasts, gastrointestinal tract, and thyroid. A 26-year-old woman who presented for replacement of her teeth, all of which had been extracted because of rapidly progressive periodontitis. She was diagnosed with Cowden's syndrome based on mucocutaneous abnormalities, thyroid involvement, and abnormalities of the skeletal and genitourinary systems. The clinical significance and differential diagnosis of this disease are highlighted.

Rheumatoid arthritis of TMJ--a diagnostic dilemma?

Rheumatoid arthritis is a chronic disease affecting the musculo-skeletal connective tissue of the body with a strong predilection for the joints. The temporomandibular joint (TMJ) is commonly affected with rheumatoid arthritis in adults and children, but it is usually among the last joints to be affected. A case report is presented of a patient undergoing orthodontic treatment who developed unilateral condylar changes and whose serum was also positive for rheumatoid factor. The involvement of TMJ in several systemic disorders is a well established fact. When such disorders manifest in an atypical manner, it poses a diagnostic dilemma. This article aims to discuss various disorders causing condylar erosions.

Prevalence of oral mucosal lesions in dental patients with tobacco smoking, chewing, and mixed habits: A cross-sectional study in South India.

BACKGROUND: A variety of oral mucosal lesions and conditions are associated with the habit of smoking and chewing tobacco, and many of these carry a potential risk for the development of cancer. There have been no studies that report the prevalence of habits and associated oral changes in the population in Dharwad region, of Karnataka, south India. MATERIALS AND METHODS: A hospital-based, cross-sectional study was carried out at SDM Dental College (Dharwad, Karnataka). A total of 2400 subjects (1200 subjects with and 1200 subjects without habits) attending the dental hospital were interviewed and examined by trained professionals to assess any oral mucosal changes. RESULTS: Oral mucosal lesions were found in 322 (26.8%) subjects who had tobacco smoking and chewing habits as compared to 34 (2.8%) subjects without those habits. Oral leukoplakia (8.2%) and oral submucous fibrosis (OSF) (7.1%) were the prevalent oral mucosal lesions found in subjects who had those habits, while the other lesions (1.7%) namely; oral candidiasis, median rhomboid glossitis, recurrent apthous ulcer, frictional keratosis, and oral lichen planus (0.9%) were frequently reported among individuals without those habits. The odds of developing oral lesions in subjects with tobacco habits was nearly 11.92 times that of abstainers (odds ratio, OR = 11.92, 95% confidence intervals, CI = 10.61-14.59%). CONCLUSION: The study showed that the risk of the development of oral lesions associated with tobacco smoking, chewing, or both is quite high. Males who had one or more of these habits showed more frequent oral changes than females. The study reinforces the association of OSF with gutkha and areca nut chewing, and leukoplakia, erythroplakia, and oral cancer with tobacco smoking, chewing, or mixed habits.

Frequency of developmental dental anomalies in the Indian population.

OBJECTIVES: To evaluate the frequency of developmental dental anomalies in the Indian population. METHODS: This prospective study was conducted over a period of 1 year and comprised both clinical and radiographic examinations in oral medicine and radiology outpatient department. Adult patients were screened for the presence of dental anomalies with appropriate radiographs. A comprehensive clinical examination was performed to detect hyperdontia, talon cusp, fused teeth, gemination, concrescence, hypodontia, dens invaginatus, dens evaginatus, macro- and microdontia and taurodontism. Patients with syndromes were not included in the study. RESULTS: Of the 20,182 patients screened, 350 had dental anomalies. Of these, 57.43% of anomalies occurred in male patients and 42.57% occurred in females. Hyperdontia, root dilaceration, peg-shaped laterals (microdontia), and hypodontia were more frequent compared to other dental anomalies of size and shape. CONCLUSIONS: Dental anomalies are clinically evident abnormalities. They may be the cause of various dental problems. Careful observation and appropriate investigations are required to diagnose the condition and institute treatment.

Trigeminal neuralgia secondary to epidermoid cyst at the cerebellopontine angle: case report and brief overview.

Trigeminal neuralgia (TN) is a paroxysmal shock-like pain restricted to the innervations of the areas of one or more trigeminal branches, often set off by light stimuli in a trigger zone. Pain attacks occur spontaneously, and can also be triggered by a nonpainful sensory stimulus to the skin, intraoral mucosa surrounding the teeth, or tongue. The pathogenesis of TN is uncertain and typically is idiopathic, but it may be due to a structural lesion. Various proposed causes include traumatic compression of the trigeminal nerve by neoplastic or vascular anomalies, infectious agents such as human herpes simplex virus, and intracranial tumors or demyelinating conditions such as multiple sclerosis. Here we report a case of a young patient diagnosed with trigeminal neuralgia secondary to epidermoid cyst at the cerebellopontine angle.