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Background: There are inconsistent reports on the prevalence of oral conditions and their associated factors among adolescents living with HIV (ALHIV). The current inconsistencies may hinder the development of clear guidelines on the prevention and treatment of oral conditions among ALHIV. This study provides an update on oral conditions and their associated factors in a cohort of South African ALHIV and receiving routine HIV treatment services at a Johannesburg HIV wellness clinic., Methods: Decayed Teeth (DT), Decayed Missing and Filled Teeth (DMFT) and Oral HIV/AIDS Research Alliance case definitions were used for caries examination and reporting of the Oral Mucosal Lesions (OML) respectively. Data analyses were stratified by the study main outcomes; chi-squared tests were performed to determine the associations; and multiple logistic regressions were also used to identify associated factors after adjusting for other exposure variables. In addition to fitting logistic regressions, we explored the data for potential confounders and effect modifiers., Results: A total of 407 ALHIV were assessed, of which 51.0% were females. The mean age of the ALHIV was 14.75 years (SD 2.43) while the median age of their parents was 43 years (IQR 37-48 years). Regardless of sex, age group and other socio-demographic characteristics, participants had high count of dental caries (DMFT>0). The overall prevalence of dental caries was 56.76% (n = 231) with mean DT score of 2.0 (SD 2.48) and mean DMFT score of 2.65 (SD 3.01). Dental caries prevalence (DT>0) was significantly associated with the HIV clinical markers. HIV RNA viral loads more than 1000 copies/ml and CD4 cell counts less than 200 count cells/mm3, increased the likelihood of having dental decay among ALHIV (p<0.05). ALHIV at WHO staging III, IV had higher caries prevalence ranging from 70% to 75% (p<0.05). The prevalence of dental caries was directly related to the presence of oral mucosal lesions (p<0.05). The prevalence of OML was 22%, with linear gingival erythema (13.8%) accounting for most of the OML. Multiple logistic regression modelling suggested that dental caries experience (DMFT>0), age category 13-15 years, WHO staging of IV and viral load of more than 1000 copies/ml significantly predicted the outcome of oral lesions as assessed using the OHARA case definitions (p<0.05). The odds of developing dental caries was also 1.5 times more among ALHIV who brush their teeth less frequently and those who reported more frequent eating of sugar sweetened diets (p<0.05)., Conclusions: There is high prevalence of dental caries and OML among ALHIV in Johannesburg. The reported prevalence was associated with high HIV RNA viral loads, shorter duration on antiretroviral treatment and high WHO staging of HIV disease on crude analysis. Additionally, caries experience contributed to the prevalence of OML. Our study acknowledges the protective effect of HIV treatment and positive oral health practices on the presence of oral conditions among ALHIV in Johannesburg., Competing Interests: The authors have declared that no competing interests exist.

This article reports on a case presenting with a rare syndrome characterized by enamel dysplasia and multiple unerupted teeth with large solid fibrous pericoronal lesions manifesting with odontogenic fibroma-like features. Our case shows in addition to these findings an anterior open bite malocclusion and gingival overgrowths. These overgrowths exhibit the microscopic features of the multiple pericoronal odontogenic fibroma-like lesions that appear to be the hallmark of this syndrome. This unusual case brings the total number documented in the literature to 5, all of which were reported from South Africa.

The increasing appearance of AIDS-associated oral Kaposi's sarcoma (KS) in South Africa may be ascribed to the later start of the HIV epidemic, more patients reaching stages III and IV, and the inaccessibility of most patients to anti-retroviral therapy. The objective of this study was to demonstrate cytomegalovirus (CMV) co-infection in oral KS and to consider its possible significance. We reviewed 20 cases of oral KS in known HIV-positive patients without active CMV disease. HHV8 PCR and CMV immunohistochemistry were performed. HHV8 DNA was present in all cases. CMV inclusions were detected in five cases. The significance of CMV co-infection in oral KS is unclear. The inclusions suggest active infection, although there is no evidence to support CMV in the pathogenesis of KS. Nonetheless, it is vital that physicians be alerted to active CMV infection, so that timely intervention and careful observation can be instituted, ensuring early diagnosis and treatment.

Background: Lipoid proteinosis (LiP) is a rare autosomal recessive disorder characterized by a hoarse voice, warty skin infiltration and scarring. Mutations within the extracellular matrix protein 1 (ECM1) gene cause LiP. Since the early 1970s it has been recognized that South Africa has one of the largest groups of LiP patients worldwide, suggesting a probable founder effect. As LiP patients present with considerable clinical variability, this group of patients offers a unique opportunity for genotype-phenotype correlation., Objectives: To assess the clinical features and the molecular basis of LiP in patients from the Namaqualand area of the Northern Cape province of South Africa and to examine molecular evidence for a founder effect., Subjects and Methods: The LiP patient cohort consisted of 29 Coloured patients from Namaqualand and a further seven Caucasoid patients from other areas of South Africa. The control group included 100 healthy geographically and ethnically matched individuals from Namaqualand. Samples were collected after informed consent and with ethics committee approval from the University of the Witwatersrand. LiP patients were examined clinically and a structured recording sheet was completed. A brief neurological evaluation was also performed. The LiP founder effect was investigated at the molecular level by ECM1 mutation detection and haplotype analysis., Results: The most consistent clinical signs for a diagnosis of LiP in this group were a hoarse voice and thickened sublingual frenulum leading to restricted tongue movement. Homozygosity for a nonsense mutation in exon 7 of the ECM1 gene, Q276X, was identified in all patients (Coloured and Caucasoid). Despite this genetic homogeneity, considerable clinical variability in skin presentation and psychiatric involvement was observed. Haplotype analysis using markers from a 9.98-Mb region around the ECM1 locus confirmed the founder effect with a founder core haplotype, 19-Q276X-12 (ND1-ECM1-D1S2343), in all but four LiP-associated alleles (n = 58). A LiP carrier rate of 1 in 9 was observed among the 100 Namaqualand controls, predicting a LiP incidence of 1 in 324 in this community., Conclusions: Although several consistent clinical features in LiP patients homozygous for the Q276X mutation in the ECM1 gene were observed, there remains considerable clinical variability. This suggests the action of genetic and environmental modifiers of disease severity. Strong molecular evidence supports a single founder effect for the high prevalence of LiP in South Africans, both Coloured and Caucasoid.

Oral lesions associated with HIV infection, as classified by the EC-Clearinghouse on Oral Problems related to HIV infection and the WHO Collaborating Centre on Oral manifestations of the immunodeficiency virus, were studied in 600 consecutive HIV-infected patients in Cape Town, South Africa. One or more lesions were seen in 60.4% of cases. Combined candidal lesions were evident in 37.8%, hairy leukoplakia in 19.7% and combined gingival/periodontal lesions in 8.5% of patients seen. Lesions less commonly recorded include oral ulceration (2.9%) and Kaposi's sarcoma (1.5%). The clinical range of lesions seen is similar to those reported elsewhere, but socio-cultural differences allowed no reliable comparison. More than a quarter of our patients had oral soft tissue discomfort necessitating treatment; in 3.3% these were the presenting symptom. This indicates a potential major public health concern requiring education in recognition and appropriate referral and management.

A 3-year longitudinal study on Heck's disease among school children was undertaken in 2 communities which are similar geographically, socioeconomically and ethnically. The variations in the annual prevalence rates (7% to 13%), the incidence rates per 1000 (38 to 68), the female to male ratios (1:0.4 to 1:1) and the average ages of the affected pupils did not differ between communities and from year to year. Lesions varied in number, size and duration. Some were seen only at one of the examinations, while others remained during the whole study. The majority were situated on the mucosae of the lower lips and cheeks. The prevalence of lesions amongst school children in these two communities was noticeably higher than that of another community of similar ethnicity studied earlier. The clinical behaviour of the lesions is in keeping with what is expected of some HPV infections. The relatively high prevalence rate and the anatomical distribution of the lesions indicate the infectious nature of the disease, which we believe is influenced to a large extent by the lifestyle in these communities.

Seventy-six cases of focal epithelial hyperplasia are encountered during an epidemiological survey in the Western Cape Province of South Africa. They affected only Cape Coloureds and were confined to pupils of reform schools and residents of a secluded community. This pattern of distribution, coupled with its typical clinical and histological features and the fact that viral particles were observed in lesions, indicates a communicable disease of viral origin, possibly a virus of the papova group. The recent discovery of its presence in South Africa indicates that it may be newly introduced into the country or that it is a new or hitheto undetected manifestation of an existing viral disease.

A retrospective study of the clinical and radiographic features of 51 nasopalatine duct cysts has been undertaken. Of these cysts 33 were in males (64.7%) and 18 in females (35.3%). The age range was 9--67 years (mean 32 years) with most cases being detected between the second and fourth decades. All of the subjects aged less than 20 years were South African Negroes and the lesions found in this group were invariably over 3.5 cm in diameter and rapidly growing. The average size for all of the cysts (after taking in 19% radiographic enlargment factor into account) was 3.5 cm in greatest diameter. The most common signs were: palatal swelling (88.2%), tooth displacement (78.4%) and sub-labial swelling (17.6%). Root resorption was found in only two of the 40 cysts which encroached upon lamina dura. No recurrences occurred following marsupialization or enucleation.

Six patients with the typical features of submucous fibrosis (SF) but without a history of betel nut chewing or an abnormal intake of chillies are described. All had palpable fibrous bands in their mouths and limited mouth opening as well as a variety of symptoms associated with SF. Biopsy specimens of the cheeks showed the classic features of SF, an atrophic epithelium and a very dense lamina propria. Also seen were hyperkeratosis, atypia and signet cell-like degeneration of the epithelium and chronic inflammation of the lamina propria. In 3 cases the fibrosis extended into the submucosa. As no reason for the disease could be demonstrated the question is asked whether people with a genetic predisposition can develop SF spontaneously.