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Florid oral manifestations in an atypical familial adenomatous polyposis family with late presentation of colorectal polyps.

Early identification and prophylactic surgery are essential in preventing development of malignancy in colorectal polyps in familial adenomatous polyposis (FAP). Previous studies indicate a 100% cumulative risk of developing colonic polyps in individuals with FAP by the age of 34 yr. However, we have previously reported late-onset and non-penetrance of polyposis in four families. We describe here in detail one of these families with florid oral manifestations detectable on panoramic radiography of the jaws, which were instrumental in the diagnosis of FAP in the index patient and for ascertainment of her family for screening. The pathological adenomatous polyposis coli (APC) gene mutation in this family was shown to segregate with dento-osseous changes. Panoramic jaw radiography is a common examination carried out prior to dental or oral surgical procedures. This case illustrates the importance of appropriate investigations, including family history and colonoscopy, even in relatively older patients with radiological findings such as those described here and in members of their families at risk of FAP.

Adenomatous Polyposis Coli↗

Oral manifestations of disseminated Cryptococcus neoformans in a patient with acquired immunodeficiency syndrome.

A palatal lesion was the initial manifestation of disseminated cryptococcosis in a patient with acquired immunodeficiency syndrome (AIDS). The diagnosis was initially verified with cytologic smears in situ and later confirmed with pathologic findings from an excisional biopsy specimen. As cryptococcosis is one of the major opportunistic infections in persons with AIDS, an increased incidence of oral manifestations of cryptococcosis can be expected.

Acquired Immunodeficiency Syndrome↗

[Multiple myeloma with oral manifestations--report of two cases].

Multiple myeloma is a neoplastic disorder of bone that originates from cells of bone marrow. It is most commonly seen within the age range of 50-80 years, cases under the age of 40 being rare. Men are affected more frequently than are women. Bone pain is the cardinal clinical symptom in multiple myeloma. Because of the destruction of bone, pathologic fracture is fairly common. In the oral manifestations, the mandible is involved far more frequently than the maxilla, especially the most active hematopoietic areas-the remus, angle and molar region of the mandible. Other signs and symptoms of jaw involvement include swelling, pain, and increased tooth mobility. Extraosseous lesions may result in paresthesia of soft tissue and gingival enlargement with bleeding tendency. Roentgenographic examination will usually reveal numerous punched-out lesions in a variety of bones. In addition, blood examination will reveal hyperglobulinemia and Bence-Jones protein may be present in the urine of myeloma patients. The histological features of myeloma are closely packed cells resembling plasma cells. Case 1 in this report is a 64-year-old female, who has been diagnosed as having multiple myeloma (IgG, lambda). She was referred to our hospital because of gingival swelling, bleeding and pain. Case 2 is a 60-year-old female suffering from spontaneous gingival bleeding. After blood, urine examination and bone marrow biopsy, multiple myeloma was diagnosed (IgG, lambda). This paper reports the clinical manifestations and treatment courses of these two cases, and the concerns of treatment of multiple myeloma are also discussed.

Female↗

Oral manifestations of Albright hereditary osteodystrophy: a case report.

Albright hereditary osteodystrophy is a hereditary metabolic disorder of dominant autosomal etiology that is commonly characterized by short stature, round face, small metacarpus and metatarsus, mental retardation, osteoporosis, subcutaneous calcification, variable hypocalcemia, and hyperphosphatemia. In this study, we report a clinical case of a 17-year-old woman with Albright hereditary osteodystrophy, and we discuss her clinical, radiographic, and laboratory test characteristics together with the oral manifestations, and we correlate them with the characteristics found in the literature. We also discuss the odontological management of treatment of related periodontal disease and planning for corrections of related malocclusions.

Adolescent↗

Granulomatous inflammation of minor salivary gland ducts: a new oral manifestation of Crohn's disease.

Multiple sublingual cystic masses developed in a 27-year-old man with active intestinal Crohn's disease during prednisone therapy. Surgical excision revealed noncaseating granulomatous inflammation involving the walls of minor salivary gland ducts. In some areas, this ductal lesion was associated with rupture and mucocele formation. Three weeks after excision of the salivary gland tissue, the patient experienced an exacerbation of his intestinal disease, accompanied by a recurrent lesion on the floor of his mouth and aphthous ulcerations of the buccal mucosa. The oral lesions and intestinal symptoms resolved only after azathioprine therapy. This salivary gland lesion represents a previously undescribed oral manifestation of Crohn's disease.

Adult↗

Oral manifestations of neurofibromatosis types I and II.

The disease known as neurofibromatosis is now recognized to consist of distinct variants that differ from each other genetically, microscopically, and clinically. Neurofibromatosis type I (NF-I) is often referred to as von Recklinghausen's disease of skin, and its features are well known. Neurofibromatosis type II (NF-II) is a much more uncommon manifestation that probably results from a structural defect in chromosome 22, as opposed to NF-I, which is related to chromosome 17. Although neurofibromas occur in NF-II, neurilemmomas and acoustic neuromas are the predominant neural tumors; bilateral acoustic neuromas are the hallmark of the disease. NF-II largely afflicts the central nervous system and has a more gradual onset than and different clinical features from NF-I. One case each of NF-I and NF-II is presented, with emphasis on oral manifestations. Diagnostic techniques, treatment, and prognosis are reviewed.

Female↗

Tuberous sclerosis: presentation of a clinical case with oral manifestations.

Tuberous sclerosis (TS) is a genetic disorder affecting multiple body systems, and resulting from alterations in cell differentiation and proliferation. The disease is characterized by the development of benign hamartomatous tumors: neurofibromas and angiofibromas, located in the skin, central nervous system, mucosas and other organs. Abnormal neural cell migration plays an important role in the neurological dysfunctions found in TS, the predominant features being mental retardation, seizures and behavioral disorders. The condition is produced by mutations in genes TSC1 of chromosome 9q34 and TSC2 of chromosome 16p13.3, and exhibits a dominant autosomal hereditary trait--though 60-70% of cases are sporadic and represent new mutations. The phenotype is highly variable. The prevalence of TS varies between 1/6000 and 1/10,000 live births. The present study reports the case of a 21-year-old male with TS and oral manifestations of the disease. The clinical characteristics are described, along with the diagnostic criteria and the management strategies, with a review of the literature on the disease.

Adult↗

Oral manifestations and differential diagnosis of isolated hypoglossal nerve palsy: report of two cases.

Isolated hypoglossal nerve palsy is rare, but occasionally it appears as the initial or solitary sign of an intracranial or extracranial space-occupying lesion, a head and neck injury, or a vascular abnormality of the internal carotid artery. Therefore it should be considered in differential diagnosis. We report two cases of isolated unilateral hypoglossal nerve palsy. In Case 1 the cause of the palsy appeared to be hypoglossal nerve neurilemmoma within the hypoglossal canal, whereas in Case 2 the cause could not be identified. Neither patient complained of any disability other than slight dysarthria. The tongue deviated toward the healthy side at rest and toward the affected side on protrusion. Hemiatrophy of the tongue with fatty displacement was demonstrated by means of T1-weighted magnetic resonance imaging. Dentists who might at times see patients with isolated hypoglossal nerve palsy should be aware of the significance of its oral manifestation, and they should be able to perform differential diagnosis of patients with the condition who appear for treatment.

Adipose Tissue↗

A new approach to managing oral manifestations of Sjogren's syndrome and skin manifestations of lupus.

Sjogren's syndrome (SS) is an autoimmune disorder that affects the salivary glands, leading to xerostomia, and the lacrimal glands, resulting in xerophthalmia. Secondary SS is associated with other autoimmune disorders such as systemic rheumatic diseases and systemic lupus erythematosis (SLE), which can affect multiple organs, including the epidermis. Recent studies have demonstrated that green tea polyphenols (GTPs) possess both anti-inflammatory and anti-apoptotic properties in normal human cells. Epidemiological evidence has indicated that, in comparison to the United States, the incidence of SS, clinical xerostomia and lupus is considerably lower in China and Japan, the two leading green tea-consuming countries.Thus, GTPs might be responsible, in part, for geographical differences in the incidence of xerostomia by reducing the initiation or severity of SS and lupus. Consistent with this, molecular, cellular and animal studies indicate that GTPs could provide protective effects against autoimmune reactions in salivary glands and skin. Therefore, salivary tissues and epidermal keratinocytes could be primary targets for novel therapies using GTPs. This review article evaluates the currently available research data on GTPs, focusing on their potential application in the treatment of the oral manifestations of SS and skin manifestations of SLE.

Anti-Inflammatory Agents↗

General and oral manifestations of human immunodeficiency virus infection: a general overview with guidelines for infection control in the dental practice.

The human immunodeficiency virus (HIV-1), responsible for the acquired immunodeficiency syndrome, has in the span of a decade become an epidemic of global proportions. Oral lesions, head and neck diseases are often the first manifestations of HIV-1 infection and AIDS. It is essential for all dentists to familiarize themselves with the oral manifestations of HIV-infection as well as the dental management of such manifestations. In addition to all health care, professionals should become acquainted with the "universal precautions" recommended by the Centers for Disease Control, in order to protect themselves, their staff, and their patients to minimize, if not totally eliminate, any risk of infectious disease transmission in the health care setting. This paper presents a general overview of the human immunodeficiency virus; oral manifestations of HIV-infection; "universal precautions" and guidelines for the control of infection in the dental practice.

HIV Infections↗

A prospective study of the oral manifestations of Crohn's disease.

BACKGROUND & AIMS: Recent studies suggest that the mouth may be involved frequently in patients with Crohn's disease (CD). The aim of this study was to document prospectively the proportion of children with oral lesions at diagnosis of CD, to describe the type of lesions found, and to examine the ability of gastroenterologists to identify correctly oral Crohn's manifestations. METHODS: In a prospective 3-year study, systematic dental examinations were performed on all children with suspected inflammatory bowel disease. Each child underwent upper endoscopy, colonoscopy, and barium follow-through radiography. RESULTS: Forty-eight of 49 children with CD were examined by the dentist. Oral CD was found in 20 patients (41.7%). Oral findings included mucogingivitis (12 patients), mucosal tags (4 patients), deep ulceration (4 patients), cobblestoning (3 patients), lip swelling (3 patients), and pyostomatitis vegetans (1 patient). Noncaseating granulomas were found in all 8 oral biopsy specimens from oral CD lesions (100%). Two patients with granulomas in oral biopsy specimens had no granulomas found in any other biopsy specimens. The presence of oral manifestations was associated with perianal disease. In only 9 patients (45%) with oral CD was the mouth found to be abnormal by the consultant gastroenterologists. Only nonspecific oral changes were seen in children with ulcerative colitis and indeterminate colitis. CONCLUSIONS: More than one third of all children presenting with CD had involvement of the mouth. The ability of physicians to recognize oral lesions was poor. Expert dental evaluation may be useful during the investigation of patients with suspected inflammatory bowel disease.

Adolescent↗