Generalized hereditary gingival fibromatosis in a child: clinical, histopathological and therapeutic aspects

Hereditary gingival fibromatosis (HGF) is a rare genetic condition characterized by slow and progressive gingival enlargement. The gingival overgrowth often delays teeth eruption and may cause serious functional and aesthetic problems. We reported a case of a 10-year-old female child presenting a generalized gingival enlargement covering almost all the maxillary and mandibular teeth and resulted in problems for swallowing, speaking, and poor aesthetics. An incisional biopsy was performed and revealed a hypocellular and hypovascular dense collagenous tissue covered by squamous epithelium exhibiting acanthosis and elongated rete ridges. The diagnosis was HGF. The treatment instituted was an association of gingivectomy with a rigorous program of oral hygiene and follow-up. Herein, we describe a rare non-syndromic case of generalized HGF, including clinical and microscopical features, as well as highlighting the importance of correct diagnosis of this genetic condition.

Immunohistochemical Analysis of Cell Proliferation and Bcl-2 Expression in Drug-Induced Gingival Overgrowth

Objective: To compare the rate of cell proliferation and expression of antiapoptotic protein Bcl-2 between drug-induced gingival overgrowth (DIGO) and clinical healthy gingiva (CHG) and to establish associations with histopathological features. Material and Methods: Twenty specimens of DIGO and 20 CHG specimens were submitted to morphological and immunohistochemical analysis by light microscopy. Cell proliferation (Ki-67) and the expression of Bcl-2 were evaluated in epithelial cells and spindle-shaped mononuclear cells of the connective tissue by establishing the labeling index (LI). Results: In epithelial tissue, the mean LI for Ki-67 was 17.2% in DIGO and 21.71% in CHG (p = 0.137). The mean LIs for Bcl-2 in epithelial tissue were 14.67% and 10.24% in DIGO and CHG, respectively (p = 0.026). In connective tissue, DIGO and CHG specimens exhibited low LIs for Ki-67 and Bcl-2, with mean values of less than 0.5% in both groups. No significant differences in the LIs for Ki-67 or Bcl-2 in epithelial tissue were observed according to the degree of collagenization, degree of vascularization and intensity of inflammatory infiltration (p > 0.05). No significant correlations were observed between the LIs for Ki-67 and Bcl-2 (p > 0.05). Conclusion: The present results suggest that the pathogenesis of DIGO does not involve increased proliferation or decreased apoptosis of fibroblasts. On the other hand, the morphological pattern of elongated epithelial cristae observed in DIGO could mainly be due to the inhibition of keratinocyte apoptosis and not to increased proliferation of these cells.

Prevalencia de lesiones benignas y desórdenes potencialmente malignos que afectan la mucosa bucal en pacientes adultos Facultad de Odontoloía UCV / Injury prevalence of benign and potencially malignant disorders affecting the mouth in patients mucosal Faculty of Dentistry UCV

Se realiza un estudio de tipo documental, retrospectivo, transversal. La población del presente estudio estuvo representada por un total de 9.000 historias diagnosticadas en el Laboratorio Central de Histopatología Bucal "Dr. Pedro Tinoco" de la Facultad de Odontología de la U.C.V., durante el período 1988-2008. La muestra evaluada quedó conformada por un total de Cuatro Mil Ciento Sesenta y Seis (4.166) casos, los cuales fueron seleccionados de manera intencional no probabilística a partir de la población antes mencionada siguiendo criterios de inclusión y exclusión. La prevalencia de Lesiones Benignas y Desórdenes Potencialmente Malignos que afectan la Mucosa Bucal es alta (46,2%) con respecto a la muestra total estudiada (9000 casos). De acuerdo al Grupo Etario observamos un intervalo de edad entre 15 y 97 años, una media de 47,3 años y Desv. Tip de 16,5 (Y ± s : 47,3 ± 16,5). En relación a la distribución por Género, en nuestro estudio existe un predominio de las lesiones de la mucosa bucal por el sexo femenino (69,7%). En base al diagnóstico clínico se identificaron 77 lesiones diferentes. El Fibroma Traumático fue la lesión más común con 1042 casos (25,01%), seguida por la Leucoplasia con 764 casos (18,33%), la Hiperplasia Fibrosa por Prótesis Dental con 447 casos (10,73%). En referencia a las diferentes localizaciones anatómicas mayormente afectadas en este estudio: el Reborde Alveolar ocupó el primer lugar (1134 casos; 27,2%), seguida por Los Carrillos (639 casos; 15,33%), Labio Inferior (522 casos; 12,5%), Encías (493 casos; 11,8%).En referencia al grado de Concordancia global entre el Diagnóstico Clínico e Histopatológico fue del 75,9 % de los casos (3.161 casos de 4.166). Situación que nos permite asumir que la concordancia global es satisfactoria.

A study of documentary, retrospective, cross. The study population was represented by a total of 9,000 stories diagnosed at the Central Laboratory of Oral Histopathology "Dr. Pedro Tinoco " in the Faculty of Dentistry at UCV, during the period 1988-2008 . The sample studied was composed of a total of Four Hundred Sixty Six Thousand (4,166 ) cases , which were selected intentionally not random from the population above following inclusion and exclusion criteria . Injury prevalence Benign and Potentially Malignant Disorders affecting Mucosa is high ( 46.2 %) compared to the total study sample (9000 cases). According to Age Group observed an age range between 15 and 97 years , an average of 47.3 years and 16.5 Desv.Tip (Y ± s : 47.3 ± 16.5). Regarding the Gender distribution in our study there is a prevalence of oral mucosal lesions in females (69.7 %). Clinical diagnoses based on 77 different lesions were identified. The Traumatic fibroma was the most common injury in 1042 patients (25.01%), followed by leukoplakia with 764 cases (18.33%), Fibrous Hyperplasia By Dental Implants with 447 cases (10.73%). Referring to different anatomical locations most affected in this study: Alveolar Flange ranked first (1134 cases , 27.2 %), followed by The Cheeks (639 cases , 15.33%), Lower Lip (522 cases; 12.5%), Gum (493 cases, 11.8%). Referring to the degree of overall concordance between the Clinical and Histopathological diagnosis was 75.9% of cases (3,161 of 4,166 cases). This situation allows us to assume that the overall agreement is satisfactory.

Fibromixoma odontogénico en el maxilar superior: reporte de un caso clínico y revisión de la literatura / Fibromixoma odontogenic in the maxilla: report of a case and literture review

El Fibromixoma Odontogénico es una variante del Mixoma Odontogénico. Se describe como una lesión intraósea agresiva derivada del tejido conjuntivo embrionario asociada con la odontogénesis1 constituida principalmente por grandes cantidades de tejido fibroso celular maduro. Su origen es controvertido, aparece en el esqueleto facial, afectando con mayor frecuencia a la mandíbula2. A continuación se presenta el caso clínico de un paciente de sexo femenino de 36 años de edad que presentó un aumento de volumen a nivel del ápice de diente 1.6 ,en la que se realizó un curetaje logrando la completa resección de la lesión, el resultado del informe patológico da el diagnóstico de Fibromixoma de origen Odontogénico

The Odontogenic Fibromyxoma is a variant of the Odontogenic Myxoma. It is described as an agressive intraoseous lesion that derives from the embrionary connective tissue associated with the odontogenesis, constituted by great amounts of celular mature fibrous tissue. It has a controverted origin, appears in the facial skeleton affecting more frecuently the mandible. We present a case of a 36 year old female who consulted with an increase of volume in relation to the 1.6 theet where we practiced a curetaje obtaining a complete resection of the lesion, the results of the patologic inform gives the diagnostic of odontogenic fibromyxoma

Minichromosome maintenance 2 and 5 expressions are increased in the epithelium of hereditary gingival fibromatosis associated with dental abnormalities

INTRODUCTION: Gingiva fibromatosis is a relatively rare condition characterized by diffuse enlargement of the gingiva, which is caused by expansion and accumulation of the connective tissue. OBJECTIVE: The aim of the present study was to investigate proliferative and apoptotic biomarker expression in normal gingiva and two forms of gingival fibromatosis. METHODS: Archived tissue specimens of hereditary gingival fibromatosis, gingival fibromatosis and dental abnormality syndrome and normal gingiva were subject to morphological analysis and immunohistochemical staining. The results were analyzed statistically. RESULTS: Proteins associated with proliferation were found in the nuclei of epithelial cells from the basal and suprabasal layers, whereas apoptotic proteins were detected in the cytoplasm of the upper layers of the epithelium. Increased expressions of minichromosome maintenance proteins 2 and 5 were observed in the gingival fibromatosis and dental abnormality syndrome samples. In contrast, geminin expression was higher in normal gingiva samples. No difference in the expression of apoptotic proteins was observed among the groups. CONCLUSION: Our findings support a role for augmented proliferation of epithelial cells within the overgrown tissues associated with gingival fibromatosis or dental abnormality syndrome. However, our data suggest that different biological mechanisms may account for the pathogenesis of different types of gingival fibromatosis.

Caso para diagnóstico / Case for diagnosis

Fibromatose hialina juvenil e hialinose sistêmica infantil são desordens autossômicas recessivas, raras da infância. À histologia, há depósito de material hialino na derme e subcutâneo. As características clínicas principais são: lesões pápulo-nodulares, hipertrofia gengival, contratura articular, lesões ósseas osteolíticas e retardo no crescimento. Mutações no mesmo gene foram identificadas nas duas condições, sugerindo que sejam espectros da mesma doença.

Juvenile hyaline fibromatosis and infantile systemic hyalinosis are rare autossomal recessive disorders with onset in infancy or early childhood. Histological examination shows deposit of hyaline material in the dermis and subcutaneous tissue. Clinical features include papulonodular skin lesions, gingival hypertrophy, flexion contractures of joints, osteolytic bone lesions and stunted growth. Mutations in the same gene were detected in both conditions, suggesting that they may be variants of the same disorder.

Análise histológica de tecido gengival de indivíduos que apresentam fibromatose gengival induzida por medicamento / Histological analysis from gingival tissue of individual with drug induce fibromatosis

O mecanismo da fibromatose gengival (FG)induzida pela ciclosporina é desconhecido, no entanto, proliferação de fibroblastos gengivais, síntese protéica e produção de colágeno parecem ser responsáveis por este efeito adverso. Objetivo: foi avaliar características da FG induzida pela ciclosporina, por meio de análise histomorfológica e histomorfométrica. Método: participaram quatro indivíduos usuários de ciclosporina (teste) e quatro periodontalmente saudáveis (controle). Amostras do tecido gengival foram obtidas após procedimento cirúrgico periodontal. Vinte e quatro lâminas foram coradas com Hematoxilina e Eosina para contagem de fibrolastos e 24 coras com Picrosírius para quantificação das fibras colágenas. Resultados: a média dos fibroblastos encontrada nos controles foi de 41,5 mais ou menos 18,7 e os testes de 58,6 mais ou mesnos 27,9. A quantificação da fração de área do colágeno foi determinada pela área ocupada em porcentagem. A médiados controles foi de 57,6 mais ou menos 7,5 e dos testes de 52,7 mais ou menos 12,2. Quando aplicado o teste t para o nível de significância de 95 por cento foi encontrada diferença estatística entre os grupos (p=0,000 e p=0,012) respectivamente. Conclusão: os achados sugerem que o mesmo apresentando uma maior quantidade de células, o volume gengival deve estar relacionado com a produção de outros componentes teciduais diferentes da produção de colágeno.

Idiopathic gingival fibromatosis. A case report.

Gingival fibromatosis is a progressive gingival enlargement caused by an over growth of the collagenous element of the gingival fibrous connective tissue. Pharmacologically induced, hereditary (familial) and idiopathic forms of gingival fibromatosis are recognized. This paper reports a case of idiopathic gingival fibromatosis in a 13 year old boy involving the right maxillary and mandibular arches who had also been treated 3 years back for a gingival enlargement involving the left maxillary and mandibular arches. The enlargement was quite sever and caused significant esthetic and functional problems on both occasions.

Fibroma de células gigantes: estudo histomorfológico de 21 casos e discussäo dos conceitos atuais / Giant cell fibroma: histomorphological study of the 21 cases and discussion of actual concepts

Foram analisados clínica e histomorfologicamente 21 casos de fibroma de células gigantes, diagnosticados no Serviço de Anatomia Patológica de Departamento de Odontologia da UFRN. Verificou-se maior ocorrência no sexo feminino (71,4 por cento) e de raça branca (66,6 por cento); a gengiva revelou-se o sítio anatômico preferencial. Microscopicamente, estas lesöes foram caracterizadas por proliferaçäo de células estreladas ou angulares volumosas, muitas delas exibindo aspecto dendrítico, ocasionalmente, contendo numerosos núcleos, e imersas em um tecido conjuntivo fibroso frouxamente arranjado. Também fotam discutidos alguns conceitos atuais acerca da histogênese desta patologia

Fibromatose gengival hereditária: revisäo e relato de um caso / The hereditary gingival fibromatosis: review and case related

Um caso de fibromatose gengival hereditária é apresentado. Com base na literatura revisada, discute-se a etiologia e caracteres clínicos, radiográficos e histopatológicos da doença. Aspectos alusivos ao tratamento também säo comentados

Hiperplasias gengivais não inflamatórias / Non-inflammatory gingival hyperplasia

Os aumentos hiperplásicos não inflamatórios da gengiva são doenças relativamente raras, podendo estar associadas a condição sistêmica de caráter hereditário, ou induzidas pelo uso de drogas anticonvulsivas do grupo da fenitoína. Independentemente da sua origem etiológica, a hiperplasia gengival apresenta um conteúdo fibroso maior que o normal, provocando problemas estéticos e dificuldades na fala e na mastigação. A inflamação observada na maioria dos casos pode ser considerada como uma condição associada à manifestação da patologia, embora alguns autores postulem que a inflamação é o resultado da formação de pseudobolsas periodontais pelo tecido hiperplásico e a conseqüente dificuldade em obter higiene bucal adequada. Além das manifestações clínicas, os aspectos histológicos, o diagnóstico e o tratamento destas hiperplasias também são discutidos no presente trabalho

Fibromatosis: acerca de nuestra casuística / Fibromatosis: a report on our experience

Se presentan tres casos de fibromatosis de comienzo infantil o juvenil: una fibromatosis digital, una fibromatosis gingival idiopática y una fibromatosis lineal progresiva. Se destaca, en el caso 1, la ubicación del proceso en el primer dedo del pie, la ausencia de cuerpos de inclusión y la no involución a los tres años de observación; asimismo, la tendencia a mejorar espontáneamente del caso 3 y la baja frecuencia con que se observan estas patologías. Se las ubica nosológicamente como intermedias entre los fibrohistiocitomas y los fibrosarcomas. Se pasa revista a las diferentes entidades descriptas en este capítulo.