Cases reported "Mouth Diseases"

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1/58. oral manifestations of Schimmelpenning syndrome: case report and review of literature.

    Schimmelpenning syndrome (SS) is characterised by specific skin manifestations, skeletal defects, and central nervous system abnormalities. Here, the SS is briefly reviewed, and the oral and dental manifestations are described in a patient whose medical findings were previously published and included severe hypophosphatemic rickets. Significant oral and dental features included papillomatous lesions of the gingiva, hemihyperplasia (hemihypertrophy) of the tongue, bone cysts, aplasia of teeth, enlarged pulp chambers, hypoplastic or absent enamel, and an odontodysplasia-like permanent tooth.
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keywords = bone
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2/58. Congenital neutropenia. Report of a case and a biorationale for dental management.

    Congenital neutropenia is characterized by a marked decrease in or lack of circulating PMN's in children with no prior history of drug intake. The neutropenia is persistent and the clinical course is one of early onset of severe, recurrent, and eventually fatal infections. bone marrow studies show a maturation arrest of neutrophilic precursors. Because of their greatly increased susceptibility to infection, patients with congenital neutropenia present a difficult dental management problem. A case of congenital neutropenia has been presented, as well as a biorationale for dental treatment. On the basis of reports in the literature, the following recommendations for the management of patients with congenital neutropenia are made: 1. The prevention and control of infection and the interception of dental disease before surgical intervention becomes necessary should be the overriding considerations in the management of patients with congenital neutropenia. 2. The carious breakdown of teeth should be prevented by the daily application of a 0.4 per cent stannous fluoride gel in addition to oral hygiene and limitation of sucrose intake. 3. Periodontal therapy should be palliative only, since alveolar bone loss is progressive despite frequent oral hygiene instruction and prophylaxis. The goal of periodontal therapy for patients with congenital neutropenia should therefore be a decrease in gingival inflammation to make the patient's mouth more comfortable and to slow down alveolar bone loss. Periodontal surgery is contraindicated. 4. bacteremia and subsequent septicemia should be prevented since a minor infection can become life threatening in patients with congenital neutropenia. The patient should rinse for 30 seconds and the gingival sulci should be irrigated with a phenolated antiseptic mouthwash prior to all dental manipulations of the soft tissue. This will significantly reduce the incidence of bacteremia. 5. Surgery should be avoided if at all possible because of the high risk of post-operative infection. All surgery sholld be performed in the hospital, and the patient should be given antibiotics as determined by his physician. Primary closure should be done with fine polyglycolic acid sutures to reduce the chance of infection. If postoperative infection can be prevented, wound healing will progress normally despite the complete absence of PMN's.
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keywords = bone
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3/58. Bacillary angiomatosis affecting the oral cavity. Report of two cases and review.

    Bacillary angiomatosis (BA) is an infectious disease characterized by proliferative vascular lesions; it mainly affects hiv-positive patients. Multiple cutaneous nodular lesions together with fever, chills, malaise, anorexia, vomiting and headache are the most important clinical manifestations. It may also involve the heart, liver, spleen, bones, lung, muscles, lymph nodes, central nervous system and other organs. erythromycin, 500 mg four times a day, is the drug of choice. The importance of this lesion lies in its clinical and histological similarity with other diseases. Cutaneous and oral lesions of BA clinically resemble Kaposi's sarcoma (KS). Histopathologically, BA may be confused with angiosarcoma, pyogenic granuloma and epithelioid hemangioma. We report two hiv-positive men with BA lesions in the oral mucosa. diagnosis was confirmed by biopsy and Warthin-Starry silver staining.
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4/58. Oral mucosal hyperpigmentation secondary to antimalarial drug therapy.

    A case of oral mucosal hyperpigmentation resulting from antimalarial drug therapy is presented. The patient reported a history of long-term quinacrine therapy and exhibited diffuse blue-gray pigmentation of the nail beds and the skin of the nasal ala. Microscopic examination of the involved mucosa showed macrophages, containing both melanin and ferric iron, scattered within the connective tissue adjacent to the epithelium. The clinical, historical, and microscopic features of antimalarial-induced pigmentation are discussed. Other causes of diffuse or multifocal oral pigmentation are also addressed.
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keywords = macrophage
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5/58. Langerhans' cell histiocytosis restricted to the oral mucosa.

    Langerhans' cell histiocytosis comprises a group of disorders with Langerhans' cell proliferation as a common feature. The clinical presentation might be highly varied. Typically, there is bone involvement and, less frequently, lesions might be found in other organs, particularly the lungs, liver, lymph nodes, skin, and mucosae. Lesions limited to the oral mucosa are rare. We describe two patients with unifocal eosinophilic granuloma exclusively limited to the oral mucosa. triamcinolone acetonide infiltration of a palatal lesion yielded a good result in one patient. Six months later, a similar lesion developed in the mandibular gingival mucosa, but it responded to the same treatment. The lesion in the other patient responded to local radiotherapy.
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keywords = bone
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6/58. Irradiation of histiocytosis X confined to the oral mucosa.

    BACKGROUND: histiocytosis X is a rare granulomatous disease and it is still under debate as to whether this disease has to be counted among malignant tumors or benign immunologic lesions. The typical localization is confined to the bone. In rare cases histiocytosis X may be localized in the oropharyngeal mucosa. patients AND methods: We report about a case of a 75-year-old woman presenting with histiocytosis X lesions solely located in the oral cavity. A total dose of 19.5 Gy was administered without any side effects. RESULTS: Two weeks after completion of irradiation a rapid regression of mucosal ulcerations and improvement of symptoms occurred. In a follow-up of 24 months the patient is still in complete remission observed. CONCLUSION: review of the literature revealed only casuistic reports about solitary lesions of histiocytosis X without bone involvement in the adulthood. External beam radiotherapy with total doses between 10 and 20 Gy seems to be effective for local control of this disease.
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keywords = bone
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7/58. dyskeratosis congenita: report of a case.

    dyskeratosis congenita is a rare multisystem condition involving mainly the ectoderm. It is characterized by a triad of reticular skin pigmentation, nail dystrophy and leukoplakia of mucous membranes. Oral and dental abnormalities may also be present. Complications are a predisposition to malignancy and bone marrow involvement with pancytopenia. The case of a 14-year-old girl is described who presented with several of the characteristic systemic features of this condition, together with the following oral features: hypodontia, diminutive maxillary lateral incisors, delayed dental eruption, crowding in the maxillary premolar region, short roots, poor oral hygiene, gingival inflammation and bleeding, alveolar bone loss, caries and a smooth atrophic tongue with leukoplakia. Although this condition is rare, dental surgeons should be aware of the dental abnormalities that exist and the risk of malignant transformation within the areas of leukoplakia.
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ranking = 2
keywords = bone
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8/58. Severe oral manifestations of chronic graft-vs.-host disease.

    BACKGROUND: Graft-vs.-host-disease, or GVHD, is the main cause of morbidity in patients who have received bone marrow transplants. Chronic GVHD, or cGVHD, occurs 100 days or more after the transplant procedure and may take the form of various oral manifestations. CASE DESCRIPTION: A 23-year-old woman received an allogeneic bone marrow transplant. Although prophylactic therapy was provided, the patient developed cGVHD. Appropriate therapy was initiated, and it received a good clinical response at all sites affected by cGVHD, except in the oral cavity. The patient received complete symptomatic relief through revised systemic therapy, improved oral hygiene, use of topical medications and a monitored diet. CLINICAL IMPLICATIONS: Effective intervention by dentists is an important part of increasing treatment effectiveness and improving quality of life in patients who received bone marrow transplants.
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keywords = bone
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9/58. Oral leishmaniasis in a hiv-positive patient. Report of a case involving the palate.

    leishmaniasis is a parasitic disease caused by a protozoon (Leishmania), with different clinical forms that are endemic in certain countries. The association of this disease in patients who are seropositive to human immunodeficiency virus (hiv) has recently been described. leishmaniasis can develop in any stage of hiv infection, although the clinical manifestations - and hence the diagnosis - tend to coincide with the periods of maximum immune depression. We present the case of a hiv-positive, ex-intravenous drug abuser (in stage B2 of the CDC, 1992) with concomitant hepatitis c infection who presented with palatinal pain and bleeding for the past 2 months. Exploration revealed a vegetating tumoration of the hard palate. hematoxylin-eosin and Giemsa staining of the biopsy confirmed the diagnosis of leishmaniasis. The definitive diagnosis was mucocutaneous leishmaniasis (MCL), for a bone marrow aspirate proved negative, and no further lesions could be established. The patient was treated with meglumine antimoniate (Glucantime), followed by improvement of the lesions.
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keywords = bone
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10/58. oral manifestations of Langerhans cell histiocytosis. Case study of a two-year-old boy.

    Langerhans cell histiocytosis is a rare pathology. It may be a single system disease at a single site or at multiple sites, or a multisystem disease; it may evolve from one of these forms into another. oral manifestations may be the first signs of the disease. Little is known as yet about its etiology. We review the nosological entity and present the case of a two-year-old boy suffering from the multisystem form, paying particular attention to the oral manifestations. physical examination on admittance revealed skin lesions in the scalp, armpits and groins, hepatosplenomegaly and bilateral otorrhea. The diagnosis was multisystem Langerhans cell histiocytosis, affecting the skin, bone, liver and CNS.
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keywords = bone
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