Lipoid proteinosis: a rare disease in pediatric dentistry

Abstract This report describes the diagnostic process of a rare disorder in a Brazilian female child. The patient presented initially as a 7-year-old with multiple whitish submucosal nodules of a fibrous consistency in the lower lip, but with an inconclusive pathology report. When she turned 9 years of age, she presented with exacerbation of the original clinical findings, which then involved the upper lip, buccal mucosa, tongue and lingual frenulum. In addition, dermatological lesions were noted on the child's limbs and face, as well as a hoarse voice. Histopathological examination of the buccal mucosa revealed dense connective tissue with hyaline foci, which were positive with periodic acid-Schiff (PAS) staining and resistant to diastase digestion. Clinical and histopathological findings led to the diagnosis of a rare genetic disease with fewer than 300 reported cases - lipoid proteinosis. Magnetic resonance imaging revealed calcium deposits in her amygdaloid region of the brain, and nasopharyngolaryngoscopy revealed lesions in her vocal cords. The patient currently is stable and under multidisciplinary follow-up, but no treatment has been recommended to date.

Resumo Este relato descreve o processo diagnóstico de uma doença rara em uma criança brasileira do sexo feminino. A paciente, inicialmente com 7 anos de idade, apresentava múltiplos nódulos submucosos esbranquiçados, de consistência fibrosa, no lábio inferior, mas com um laudo patológico inconclusivo. Quando completou 9 anos de idade, ela apresentou exacerbação dos achados clínicos originais, que envolveram o lábio superior, mucosa bucal, língua e frênulo lingual. Além disso, lesões dermatológicas foram observadas nos membros e no rosto da criança, assim como rouquidão. O exame histopatológico da mucosa bucal revelou tecido conjuntivo denso com focos hialinos, que foram positivos com coloração periódica com ácido-Schiff (PAS) e resistente à digestão da diástase. Os achados clínicos e histopatológicos levaram ao diagnóstico de uma doença genética rara com menos de 300 casos relatados - proteinose lipoide. A ressonância magnética revelou depósitos de cálcio em amígdala cerebral e a nasofaringolaringoscopia revelou lesões em cordas vocais. Atualmente, a paciente está estável e em acompanhamento multidisciplinar, mas nenhum tratamento foi recomendado até o momento.

Oral Candida spp carriage and periodontal diseases in HIV-infected patients in Ribeirão Preto, Brazil

ABSTRACT The majority of HIV-infected patients develop Candida spp-associated clinical oral lesions. Studies have shown that asymptomatic oral colonization of Candida spp may lead to oral lesions or become a source of disseminated infections. The aim of this study was to verify the effects of periodontal conditions on Candida spp prevalence and Candida spp carriage in the oral cavity of HIV-infected patients compared to non-infected patients. Twenty-five patients not infected with HIV and 48 HIV-infected patients were classified according to periodontal conditions as being periodontal healthy or with periodontal disease. Candida spp carriage and classification were performed in oral rinse samples. Viral load and CD4+ T lymphocyte (CD4+L) counts were performed in blood samples from HIV-infected patients. No differences in Candida spp prevalence related to HIV status or periodontal condition were detected. However, Candida spp carriage was increased in periodontally affected HIV-infected patients when compared to periodontally healthy HIV-infected patients (p= 0.04). Periodontally healthy HIV-infected patients presented Candida spp carriage in similar levels as healthy or periodontally affected non-HIV-infected patients. Candida spp carriage was correlated with CD4+L counting in HIV-infected patients. We concluded that periodontal disease is associated with increased Candida spp carriage in HIV-infected patients and may be a predisposing factor to clinical manifestations of candidiasis.

Oral health management of 97 patients living with HIV/AIDS in Ribeirão Preto, São Paulo, Brazil

Considering the changes antiretroviral therapy (ART) has brought to the treatment of HIV infection, the current clinical and laboratory profiles of HIV/AIDS individuals referred to oral health centers are crucially important in instructing dentists about the oral health management of these patients. The aim of the present study was to determine the clinical and laboratory profiles of HIV-infected individuals referred to a clinic for patients with special needs between 2005 and 2012 by retrospectively analyzing their dental records. A total of 97 records of HIV patients referred to the School of Dentistry of Ribeirão Preto, Universidade de São Paulo - USP, were analyzed. The Mann-Whitney test was used to determine the associations between mean CD4+ counts, mean viral load, and the presence of HIV-related oral lesions (HIV-OL). Most of the patients were male, and their mean age was 38.3 years. Eighty-nine (92%) patients were on regular ART, 77 (79.4%) had a CD4+ count higher than 200 cells/mm3, and 63 (64.9%) had an undetectable viral load. Twenty patients (20.6%) presented with some HIV-OL, including pseudomembranous and/or erythematous candidiasis and angular cheilitis, which were correlated with a low CD4+ count and with an undetectable viral load (p < 0.05). Among the branches of dentistry, periodontics, followed by surgery and restorative dentistry, was the most sought-after specialty, and no intercurrent events were observed during the dental treatment. It may be concluded that there are no restrictions on the dental treatment of patients on regular ART, It is important, though, that the treatment be based on local characteristics and on the prevention of oral diseases.