Oral and craniofacial findings in Fabry's disease: a report of 13 patients.

OBJECTIVE: Fabry's disease is an X-linked metabolic disease caused by the deficiency of the lysosomal enzyme alpha-galactosidase A. The purpose of this study was to assess oral and craniofacial findings in a cohort of patients with Fabry's disease to facilitate recognition of this condition and early treatment of its manifestations. STUDY DESIGN: This is a case series describing oral and craniofacial findings of 13 male patients diagnosed with Fabry's disease. Data were collected by means of a standardized questionnaire, clinical examination, panoramic and cephalometric radiographs, and magnetic resonance imaging. RESULTS: A variety of abnormalities are described, including an increased prevalence of cysts/pseudocysts of the maxillary sinuses (PCMs) and the presence of maxillary prognathism. CONCLUSION: Given the high prevalence of oral and dental abnormalities, we recommend a thorough stomatologic evaluation of these patients.

Analgesic effects of peripherally administered opioids in clinical models of acute and chronic inflammation.

A series of double-blind, placebo-controlled clinical trials demonstrated that low doses of morphine (0.4, 1.2, and 3.6 mg) administered into the intraligamentary space of a chronically inflamed hyperalgesic tooth produced a dose-related naloxone-reversible analgesia. This analgesic effect is mediated by a local mechanism in the inflamed tissue, because subcutaneous administration of a 1.2 mg dose of morphine failed to elicit an analgesic response. In contrast, submucosal administration of 1.2 mg morphine or 50 microg fentanyl to the site of extraction of an impacted third molar after the onset of acute pain failed to elicit an analgesic response despite demonstration of a sensitive bioassay. These data indicate that peripheral opioid analgesia can be evoked in a model of chronic, but not acute, inflammatory pain, suggesting a temporal dependent mechanism needed for the expression of peripheral opiate analgesia during inflammation in humans.

Small proline-rich protein 1 is the major component of the cell envelope of normal human oral keratinocytes.

Oral keratinocytes of buccal and gingival tissues undergo a terminal differentiation program to form a protective epithelial barrier as non-keratinized or parakeratinized stratified cells. We have examined the protein composition of cell envelopes (CEs) from normal human buccal and gingival tissues as well as keratinocytes from normal human gingival cells grown in culture. Biochemical and sequencing analyses reveal that the CEs contain 60-70% small proline-rich protein 1a/b (SPR1a/b), together with smaller amounts of involucrin, annexin I and several other known CE proteins. The data imply a specialized role for SPR1 proteins in the unique barrier function requirements of oral epithelia.

Bilateral condylar resorption in dermatomyositis: a case report.

Polymyositis is an inflammatory disease commonly affecting the striated muscle. When it is accompanied by characteristic skin lesions, the condition is called dermatomyositis. Bilateral condylar resorption has been reported with autoimmune conditions and chronic systemic steroids. We report the first documented case of bilateral condylar resorption in a patient with dermatomyositis. Possible etiologic factors and treatment outcomes are discussed.

The mouth is a gateway to the body: gene therapy in 21st-century dental practice.

Gene therapy may become an integral tool in dental practice early in the 21st century. It and other biological therapies are expected to be applied to oral diseases and disorders during the midpractice lifetime of today's dental students. If the applications of oral gene transfer are expanded to systemic diseases, oral health care providers in the future could routinely be "gene therapists" with therapeutic targets well outside the oral cavity.

Nevoid basal cell carcinoma syndrome with medulloblastoma in an African-American boy: a rare case illustrating gene-environment interaction.

We present an 8-year-old African-American boy with medulloblastoma and nevoid basal cell carcinoma syndrome (NBCCS) who exhibited the radiosensitive response of basal cell carcinoma (BCC) formation in the area irradiated for medulloblastoma. Such a response is well-documented in Caucasian NBCCS patients with medulloblastoma. The propositus was diagnosed with medulloblastoma at the age of 2 years and underwent surgery, chemotherapy, and craniospinal irradiation. At the age of 6 years, he was diagnosed with NBCCS following his presentation with a large odontogenic keratocyst of the mandible, pits of the palms and soles and numerous BCCs in the area of the back and neck that had been irradiated previously for medulloblastoma. Examination of other relatives showed that the propositus' mother also had NBCCS but was more mildly affected; in particular, she had no BCCs. This case illustrates complex gene-environment interaction, in that increased skin pigmentation in African-Americans is presumably protective against ultraviolet, but not ionizing, radiation. This case and other similar cases in the literature show the importance of considering NBCCS in the differential diagnosis of any patient who presents with a medulloblastoma, especially before the age of 5 years, and of examining other close relatives for signs of NBCCS to determine the patient's at-risk status. Finally, for individuals who are radiosensitive, protocols that utilize chemotherapy in lieu of radiotherapy should be considered.

Embryonal rhabdomyosarcoma of the floor of the mouth. A case report.

Rhabdomyosarcoma is a neoplasm composed of striated muscle. One of the most common types of soft tissue mesenchymal tumors in infants, children, and adolescents, rhabdomyosarcoma was considered an almost uniformly fatal cancer as recently as 30 years ago. Presently a multimodal approach consisting of surgery, radiation, and chemotherapy has significantly improved the outlook for the patient with rhabdomyosarcoma. This article presents a case of intraoral rhabdomyosarcoma that was initially poorly controlled because of a lack of patient compliance. After proper treatment, the cancer went into remission. The patient remained cancer-free for 7 years, albeit with significant morbidity. This case demonstrates the significantly improved prognosis achieved with multimodal therapy.

Metastatic malignant melanoma of the oral cavity. A retrospective study.

Metastatic malignant melanoma of the oral cavity is rarely reported in the dental literature. This retrospective study identified metastatic oral lesions in 3.0% of 809 patients with melanoma treated at the National Institutes of Health between 1953 and 1989. Fifteen cases met established rigorous criteria for metastatic tumors and were reviewed for disease course and outcome. Nine white men and six white women, with an average age of 40.6 years, had cutaneous primary tumors predominantly of the trunk and head and neck region that commonly presented as moles that were enlarging, bleeding, or showing both of these signs. A mean of 4.2 years elapsed between primary tumor and oral metastasis diagnosis. Tongue, buccal mucosa, and parotid gland were the predominant oral sites. Enlarging oral masses, pigmented lesions, and nonhealing extraction sites with masses were common oral presentations. Surgery, chemotherapy, radiation therapy, and immunotherapy were used in various combinations for treatment of the primary lesion and oral metastasis. Prognosis, although poor, was highly variable.

Comparison of single-dose ibuprofen lysine, acetylsalicylic acid, and placebo for moderate-to-severe postoperative dental pain.

In a single-dose, double-blind, parallel-group, single-site study, ibuprofen lysine 200 mg (IBL 200) was compared with acetylsalicylic acid 500 mg (ASA 500) and placebo in 183 patients with moderate-to-severe postoperative dental pain. The relative onset of analgesic response, duration and degree of analgesia, and safety were assessed over a 6-hour postdose period. Analgesic efficacy was assessed by patient self-rating of pain intensity, pain relief, time to meaningful pain relief, global evaluation, and requirement for additional analgesic medication; both IBL 200 and ASA 500 were significantly more effective than placebo. IBL 200 also had a significantly faster onset of action, greater peak and overall analgesic effect, and longer duration of analgesia than ASA 500. All treatments were generally well tolerated.

Body and orofacial cathexis in edentulous complete-denture-wearing clients.

This description cross-sectional study examines cathexis, or satisfaction, with the body and the orofacial region in relationship to eight major demographic and health status variables in a nonprobability sample of 34 male and 47 female edentulous subjects aged 36 to 77 years. Kiyak's 27-item Body Cathexis Scale (BCS), including an innovative five-item Orofacial Cathexis Scale (OFCS), was shown to be reliable for use in elderly clients at least to age 77 (Cronbach's alpha = .95). More research is needed to validate the OFCS. Overall scores were in the neutral range, with teeth being the most negatively cathected body part. Longer periods of edentulism were associated with more dissatisfaction with the teeth. Body and orofacial cathexis were highly positively correlated. Chronic illness, education level, race, and religion were not significantly related to cathexis. Edentulism, especially recent edentulism in the elderly, female gender, and divorced marital status could be important indicators for body image disturbance.

Human immunodeficiency virus (HIV) and HIV infected cells in saliva and salivary glands of a patient with systemic lupus erythematosus.

A young woman with systemic lupus erythematosus (SLE) was infected with human immunodeficiency virus 1 (HIV-1) and about 6 years later developed persistent bilateral parotid gland enlargement. It was unclear whether this represented salivary gland involvement as a component of her SLE (secondary Sjögren's syndrome) or the initial clinical manifestation of her HIV-1 infection. HIV proviral DNA was found in individual salivary glandular secretions and in whole saliva. Additionally, cells positive for HIV RNA were isolated from whole saliva. A parotid gland biopsy revealed infiltrating lymphocytes containing large amounts of HIV RNA.

Implant rehabilitation in Erdheim-Chester disease: a clinical report.

Successful osseointegration of endosseous titanium implants is thought to be dependent upon close apposition of bone to the implant surface. The integration of implants in this patient was achieved despite the lipid-laden histiocytic infiltration of the bone marrow. Presumably, enough unaffected stromal cells were present to allow sufficient bone formation for osseointegration of the implant fixtures. This result invites speculation regarding both the mechanism of osseointegration and the minimum surface area of bone-implant interface necessary for achieving and maintaining osseointegration of titanium implants. This patient is periodically examined to determine if the loaded fixtures will remain clinically immobile for a prolonged period.

Using endosseous dental implants for patients with ectodermal dysplasia.

Congenitally missing teeth and poorly developed or absent alveolar ridges are signs often associated with various types of ectodermal dysplasia. Endosseous dental implants may be used to support fixed mandibular prostheses in patients with ectodermal dysplasia. Anatomical factors and age considerations require careful attention to treatment planning.

Mandibular osteomyelitis in a patient with sickle cell anemia: report of case.

A case is presented in which mandibular osteomyelitis and mental nerve paresthesia developed in a patient with sickle cell anemia. This infection appeared to precipitate a sickle cell crisis. The mechanisms for these patients' propensity to infection and the diagnosis and management of sickle cell osteomyelitis of the jaws are discussed.

Oral squamous cell carcinoma arising in a patient with long-standing lichen planus. A case report.

The risk of malignant transformation of oral lichen planus remains a controversial point. Many previous reports have been discounted on the basis of inadequate information or lack of histologic confirmation of lichen planus. We report a well-documented case of long-standing cutaneous and oral lichen planus in which squamous cell carcinoma of the dorsal portion of the tongue occurred. There is an apparent difference in the sites of oral carcinomas in patients with lichen planus compared with the general population. This suggests that lichen planus increases the risk of oral cancer in affected sites.

Oral manifestations of human immunodeficiency virus infection.

It is important that both physicians and dentists recognize the earliest signs of HIV infection in order that a timely diagnosis and patient referral can be made for counseling and treatment. Candidiasis, hairy leukoplakia, and Kaposi's sarcoma are the most common oral manifestations, but there are other important lesions as well. They include severe necrotizing periodontitis, bacterial and viral infections, lymphomas, and carcinomas. The various oral lesions seen in patients with the acquired immunodeficiency syndrome are reviewed and managements are discussed.

Failure of surgery and isotretinoin to relieve jaw immobilization in fibrodysplasia ossificans progressiva: report of two cases.

Two patients with fibrodysplasia ossificans progressiva who presented with jaw immobilization due to formation of bone between the maxilla and mandible were treated with surgical resection of their ectopic bone in conjunction with experimental, adjunctive medical therapy using isotretinoin. Both patients had recurrence of their ectopic ossification within 2 months of surgery. Surgery to remove joint-bridging ossifications in FOP is not recommended.

Oral manifestations of AIDS: a study of 84 patients.

Early diagnosis of AIDS is vital in providing appropriate referral for medical care and counseling. Subtle intraoral signs and symptoms identified by the dental practitioner can aid in the diagnosis of the disease. Because the role of the practitioner in detection and treatment of AIDS patients is becoming greater, signs found in 84 patients are described.