1/51. Gingival fibromatosis combined with cherubism and psychomotor retardation: a rare syndrome. Gingival fibromatosis is frequently an isolated condition, but rarely associated with some uncommon syndromes. This paper describes an 11-year-old patient with pronounced gingival enlargement, cherubic facial appearance, and psychomotor retardation and discusses the major aspects of the case. The most striking finding orally was the presence of grossly hyperplastic gingiva, which completely covered all teeth except the occlusal surfaces of some teeth. The swelling in the lower part of the face and the appearance of sclera beneath the iris suggest cherubism. The diagnosis was confirmed by the detection of giant cell regenerative granuloma and perivascular eosinophilic particles and osteoclasts after biopsy of the mandible. In this case, surgery was the only effective way to treat the patient. A full-mouth gingivectomy procedure was performed under general anesthesia in 2 stages. The case was followed for 12 months and no recurrence was seen. An appropriate oral hygiene regimen was established. ( info) |
2/51. Unusual peripheral odontogenic tumors in the differential diagnosis of gingival swellings. Differential diagnosis of gingival mass lesions includes several conditions and causes. Peripheral odontogenic tumors may mimic gingival swellings and, although rare, must be included in the differential diagnosis. The purpose of this article is to describe 3 different cases of peripheral odontogenic tumors and to discuss the differential diagnosis of gingival swelling. Histologic examination is mandatory when localized gingival swellings are surgically removed. ( info) |
3/51. Recurrent "non-Hodgkin's lymphoma presenting with gingival enlargement. Report of a case. A 66-year-old white woman with recurrent "non-Hodgkin's lymphoma" is discussed. Her initial manifestation of recurrent disease was gingival enlargement. We reviewed the oral manifestations of lymphoma and discussed the course of this patient's disease, with particular attention to the local treatment of the gingival enlargement. ( info) |
4/51. Congenital generalized fibromatosis. An African case with gingival hypertrophy and other unusual features. What is believed to be the first reported case of congenital generalized fibromatosis in an African infant is described. Features in our patient, which were not noted in previous reports of the disease, include gingival hypertrophy, ankylosis of joints, skeletal hyperostosis, and lymphatic dilation of the ileal villi. Corticosteroid therapy was tried in the patient, but did not produce any beneficial effect. ( info) |
5/51. syndrome of gingival hypertrophy, hirsutism, mental retardation and brachymetacarpia in two sisters: specific entity or variant of a described condition? Two sisters born to consanguineous Lebanese parents had mental retardation and epilepsy, brachymetacarpalia, hirsutism, bulbous soft nose, thick floppy ears with abnormal configuration and gingival hypertrophy. One girl presented additionally with tetralogy of fallot and the other with congenital hypothyroidism and bilateral ureteral stenosis. These manifestations resemble the syndrome of hypertrichosis-gingival fibromatosis-mental retardation and seizures of Anavi et al. [1989: Dev Med child Neurol 31:538-542] but our two girls additionally have brachymetacarpia. The inheritance seems to be autosomal recessive. These two sisters may represent a hitherto undescribed syndrome. We discuss the findings in our patients in relation to the literature. ( info) |
| This report is concerned with the manifestations associated with focal dermal hypoplasia (Goltz syndrome) in two patients. Goltz syndrome is a rare polydysplastic syndrome. Systemic findings were similar to those previously reported in the literature and are illustrated. More detailed descriptions of the dental abnormalities are presented. The possibility of root resorption being a previously unreported part of the syndrome is hypothesised. ( info) |
7/51. Juvenile hyaline fibromatosis complicated with oral squamous cell carcinoma: a case report. A 45-year-old woman was referred because of swelling of the palate, gingival hypertrophy, and multiple cutaneous tumors. She had many cutaneous tumors, which covered most of her body, and she also displayed contractures of the major joints. Maxillary and mandibular gingival hypertrophy, malposition of the teeth, and swelling of the hard palate were the oral findings. The histopathologic features of the cutaneous and gingival tumors were consistent with hyaline fibromatosis, and the swelling of the palate proved to be a squamous cell carcinoma. The carcinoma was treated with tegafur/uracil and seemed to respond to this therapy. ( info) |
8/51. epidermolysis bullosa acquisita: clinical manifestations, microscopic findings, and surgical periodontal therapy. A case report. BACKGROUND: epidermolysis bullosa acquisita (EBA) is an uncommon, acquired, chronic subepidermal bullous disease. This report describes a case of EBA with gingival involvement. A 43-year-old woman with EBA was referred to our clinic for periodontal therapy because of gingival tenderness and bleeding. She has been on cyclosporin A therapy for the last 2 years. methods: Clinical findings were analyzed. Anterior gingivectomy operations were performed in 2 stages. The samples obtained during the surgery were examined using histopathologic, immunohistologic, and electronmicroscopic methods. Long-term effects of the surgical periodontal treatment on gingiva were evaluated both clinically and microscopically. RESULTS: The dentition displayed minimal enamel hypoplasia. Decayed, missing, and filled surfaces score was found to be elevated. Periodontal examination showed generalized diffuse gingival inflammation and gingival enlargement localized mainly to the anterior region. Nikolsky's sign was positive. However, wound healing was uneventful after the operations. Microscopic findings were similar to those obtained from the skin. Twenty-one months after the operations, Nikolsky's sign was negative and no remarkable gingival inflammation was noted. Microscopic examination revealed that the blisters were fewer in number and smaller in size. CONCLUSIONS: These results indicate that gingival tissues may also be involved in EBA. Uneventful wound healing after periodontal surgery in this case suggests that periodontal surgery can be performed in patients with EBA. Moreover, both our clinical and histopathologic findings imply that gingivectomy proves useful in maintaining gingival integrity in these patients. Our data may also suggest that the patients with EBA are highly likely to develop dental caries. ( info) |
9/51. Dracula's teeth syndrome. OBJECTIVE: To describe a patient with facial pain, ipsilateral facial dystonia, and phantom supernumerary teeth, beginning after resection of hypertrophic gums. She had familial sensorineural deafness. BACKGROUND: Atypical cranial dystonia subsequent to dental procedures is associated with facial pain, dysesthesias, or phantom phenomena, but not with phantom supernumerary teeth. patients with migraine are susceptible to experience cephalic phantom phenomena after dental procedures. Wynne syndrome is a hereditary autosomal dominant disorder characterized by congenital sensorineural deafness and supernumerary teeth. methods: Clinical examination, computerized tomography of paranasal sinuses and facial bones, magnetic resonance imaging of the brain, cranial electrophysiological testing, and electroencephalogram. RESULTS: A 52-year-old woman with history of migraine without aura for 40 years exhibited focal right facial involuntary tonic contracture accompanying chronic severe pain over the same area after gum resection. She reported a daily sensation of having two extra upper canine teeth pressing on her tongue, simulating vampire's ("Dracula's") teeth. She had high-frequency bilateral sensorineural deafness. Her computerized tomography studies, brain magnetic resonance imaging, and cranial electrophysiological testing were normal. CONCLUSION: This patient with chronic migraine had atypical cranial dystonia beginning after a dental procedure. Her dystonia was complicated by the unusual phenomenon of phantom supernumerary teeth. This condition may be misdiagnosed as atypical or psychogenic facial pain when facial dystonia is localized or subtle and is, therefore, confused with an idiosyncratic gesture or habitual spasm. Her signs and symptoms are reminiscent of Wynne syndrome. ( info) |
10/51. Periodontal lesions in lipoid proteinosis. Lipoid proteinosis is a rare autosomal recessive heritable disease. Clinical features are hoarseness starting in infancy and the formation of yellowish papules and plaques on the skin and mucous membranes of the nose, oral cavity, pharynx and larynx. light and electron microscopy demonstrates a subepithelial accumulation of PAS-positive (hyaline) material around blood vessels and in the dermis. This case is unusual in that the gingiva is also involved in the lipoid proteinosis. ( info) |