ABSTRACT: Mucous membrane pemphigoid (MMP) is a blistering disease that predominantly involves the mucous membranes and that can lead to major negative impacts on patient quality of life. The first-line MMP treatment is based on the use of topical and systemic corticosteroids. In this report, we presented a 45-year-old female patient presented blisters in the inferior gingiva for over 8-months. The patient reported being allergic to corticosteroids. Under the clinical hypothesi s of oral lichen planus and MMP, an incisional biopsy was performed, and the histopathological diagnosis of MMP was established. Thus, it was instituted an alternative therapy with tacrolimus 0.03 %. The patient showed an excellent clinical outcome with no recurrence five months after the end of therapy. Tacrolimus 0.03 % may represent an effective therapeutic alternative in MMP treatment and may be used in cases of hypersensitivity to standard therapy.

RESUMEN: El penfigoide de la membrana mucosa (PMM) es una enfermedad ampollosa que afecta predominantemente a las membranas mucosas y que puede provocar importantes impactos negativos en la calidad de vida del paciente. El tratamiento de primera línea de PMM se basa en el uso de corticosteroides tópicos y sistémicos. En este informe, presentamos un caso de una paciente femenina de 45 años que presentó ampollas en la encía inferior durante más de 8 meses. La paciente informó ser alérgica a los corticosteroides. Bajo la hipótesis clínica de liquen plano oral y PMM, se realizó una biopsia incisional y se estableció el diagnóstico histopatológico de PMM. Por lo tanto, se instituyó una terapia alternativa con tacrolimus tópico al 0,03 %. La paciente mostró un excelente resultado clínico sin recurrencia después de 5 meses de la terapia final. Tacrolimus 0,03 % puede representar una alternativa terapéutica efectiva en el tratamiento de PMM y se puede usar en casos de hipersensibilidad a la terapia estándar.

ABSTRACT: Stafne bone defect (SBD) is a bone cavity in the lingual surface of the mandible normally filled by salivary gland tissue. In conventional radiographs, SBD typically resembles a radiolucent unilocular lesion with welldefined margins, localized under the inferior alveolar canal. The diagnosis of SBD is often incidental due to the asymptomatic nature. The aim of this study was to investigate the prevalence of SBDs in a Brazilian population and to describe the radiographic features of the cases reported. This retrospective study evaluated 17,180 digital panoramic radiographs of patients with an indication of radiography for dental treatment seen at three centers located in the three Brazilian states. In each center, two researchers evaluated the images for establishment of the consensual diagnosis of SBD. In the case of disagreement, a third researcher was consulted to reach a final consensus. To assess the prevalence of SBDs, sex and age of patients were considered, and SBDs were classified according to their form and location. Data were submitted to descriptive analysis. Among the 17.180 patients, only 15 (0.08 %) had SDB, including 3 women and 12 men. The age range of the patients with SDB was 30-69 years (mean: 49.2). Fourteen cases were located in the posterior region of the mandibular body and one case in the ascending ramus. Stafne bone defect is a rare developmental anomaly that more commonly affects middle-aged men. The condition has a typical radiographic appearance and panoramic radiography is a valuable tool for its diagnosis.

RESUMEN: El defecto óseo de Stafne (DOS) es una cavidad ósea en la superficie lingual de la mandíbula, normalmente llena de tejido glandular salival. En las radiografías convencionales, el DOS generalmente se asemeja a una lesión unilocular radiotransparente con bordes bien definidos, ubicada debajo del canal alveolar inferior. El diagnóstico de DOS a menudo es accidental debido a su naturaleza asintomática. El objetivo de este estudio fue investigar la prevalencia de DOS en una población brasileña y describir las características radiográficas de los casos reportados. Este estudio retrospectivo evaluó 17.180 radiografías panorámicas digitales de pacientes con indicación radiográfica para tratamiento dental atendidos en tres centros ubicados en tres estados brasileños. En cada centro, dos investigadores evaluaron las imágenes para establecer un diagnóstico consensuado de DOS. En caso de desacuerdo, se consultó a un tercer investigador para llegar a un consenso final. Para evaluar la prevalencia de DOS, se consideraron el sexo y la edad de los pacientes, y se clasificaron según su forma y ubicación. Los datos fueron sometidos a análisis descriptivo. Entre los 17.180 pacientes, solo 15 (0,08 %) tenían DOS, incluidos 3 mujeres y 12 hombres. El rango de edad de los pacientes con DOS fue de 30 a 69 años (media: 49,2). Catorce casos se ubicaron en la región posterior del cuerpo mandibular y un caso en la rama ascendente. Los defectos óseos de Stafne son una anomalía rara del desarrollo que afecta más comúnmente a los hombres de mediana edad. La condición tiene una apariencia radiográfica típica y la radiografía panorámica es una herramienta valiosa para su diagnóstico.

Abstract: BACKGROUND: Recurrent aphthous ulcerations are common benign ulcerated lesions on the mouth, whose etiology is poorly understood, with controversial treatment and difficult to control in clinical practice. OBJECTIVE: To evaluate the cases of recurrent aphthous ulcerations with a focus on treatment, diagnosis and etiology. METHODS: This is a retrospective study of the cases of the Oral Diagnosis service of the Rio Grande do Norte Federal University in Natal/RN. Data such as sex, age, race, location, smoking habits, types of treatment, relapsing episodes, laboratory test results and clinical characteristics were collected. The associations between the variables were analyzed using the Pearson Chi-square test (p <0.05). RESULTS: A total of 4895 patients were seen in the service over a period of 11 years. Of these, 161 (3.3%) had complaints of oral aphthous ulcerations, of which 76 (47.2%) were diagnosed as suffering from recurrent aphthous ulcerations and 68 (42.2%) with clinical information necessary for evaluation. The tongue was the most affected anatomical region, with 27 individuals (39.7%), followed by the buccal mucosa, with 22 cases (32.3%). STUDY LIMITATIONS: Retrospective study with data from medical records. CONCLUSION: Dental surgeons, dermatologists and otorhinolaryngologists are the main responsible for the first contact with patients with this disease and should be attentive to the clinical aspects and treat each patient in an individualized way, since the therapy is palliative, its diagnosis is by exclusion and its etiology is unknown.

RESUMO Objetivo: Realizar um estudo retrospectivo em uma série de casos de PV e BMMP com manifestações em cavidade oral com o intuito de verificar suas prevalências, características sociodemográficas, diagnóstico, tratamento e acompanhamento. Métodos: Trata-se de um estudo retrospectivo descritivo no qual os dados clínicos foram coletados a partir dos prontuários de todos os casos de PV e BMMP registrados e diagnosticados, entre 1995 e 2015, no serviço de Diagnóstico Oral do Departamento de Odontologia da UFRN. Resultados: Da amostra total (n=36), a age média foi de 41,64 anos, sendo o sexo female o mais frequente (n=26; 72,22%) e a mucosa jugal o sítio mais afetado (n=20; 27,40%). Oito pacientes (22,22%), dos quais 5 são casos de PV e 3 de BMMP, foram reavaliados clinicamente. Todos os pacientes exibiram lesões no momento do acompanhamento, sendo a prednisona (n=7) e o propionato de clobetasol (n=8) os fármacos mais utilizados no tratamento sistêmico e tópico, respectivamente. O tempo de acompanhamento variou de 5 meses a 5 anos. Conclusão: O perfil clínico dos pacientes da presente pesquisa foi semelhante ao da literatura. No entanto, verificou-se que as lesões orais foram mais resistentes ao tratamento empregado nos pacientes avaliados.

ABSTRACT Objective: To conduct a retrospective study on a series of cases of PV and BMMP with manifestations in the oral cavity in order to ascertain prevalence, sociodemographic characteristics, diagnostic maneuvers, treatment and follow-up. Methods: This is a retrospective, descriptive study in which clinical data were collected from the medical records of all cases of PV and BMMP registered and diagnosed, between 1995 and 2015, in the Oral Diagnostic Service of the UFRN Department of Dentistry. Results: The mean age of the total sample (n = 36) was 41.64, with females the most frequent (n = 26; 72.22%) and the cheek mucosa being the site most affected (n = 20; 27.40%). Eight patients (22.22%), including 5 cases of PV and 3 BMMP, were clinically reevaluated. All patients exhibited lesions at the time of follow-up. Prednisone (n=7; 87.5%) and clobetasol propionate (n=8, 100%) were the most widely used drugs in the systemic and topical treatment, respectively. The follow-up period ranged from 5 months to 5 years. Conclusion: The clinical profile of patients in this study was similar to that evidenced in the literature. However, it was found that the oral lesions were more resistant to the treatment used on the patients evaluated.

Resumo O angioleiomioma é uma neoplasia benigna que, a partir da nova classificação da OMS (2013) para os tumores de tecidos moles, deixou de ser considerado um tumor de origem muscular lisa, passando a ser considerado um tumor de origem perivascular. Raramente os angioleiomiomas ocorrem na cavidade oral. A lesão é tratada cirurgicamente, com prognóstico considerado favorável. Este trabalho revisa os casos de angioleiomioma oral relatados na literatura nos últimos 5 anos e descreve esse tumor em um homem de 44 anos que apresentou um nódulo assintomático localizado em lábio superior, com evolução de 6 meses. As hipóteses diagnósticas foram de adenoma pleomórfico e adenoma canalicular. A lesão foi submetida à biópsia e análise histopatológica e imuno-histoquímica (S100, CD34, α-SMA, H-caldesmon e desmina) confirmaram o diagnóstico de angioleiomioma. Destacamos a imuno-histoquímica como um importante método auxiliar no diagnóstico diferencial do angioleiomioma com outras lesões e, principalmente, com o miopericitoma.

Abstract Angioleiomyoma is a benign neoplasm that was considered a tumor of smooth-muscle origin until the most recent (2013) WHO classification of soft tissue tumors, in which it was reclassified as a tumor of perivascular origin. Angioleiomyomas rarely occur in the oral cavity. These lesions are treated surgically with good prognosis. This article presents a review of reports of oral angioleiomyoma in the literature from the last 5 years and describes the case of a 44-year-old man who presented with an asymptomatic nodule in the upper lip that had developed over a 6-month period. Diagnostic hypotheses of pleomorphic adenoma or canalicular adenoma were raised. Biopsy of the lesion, histopathological and immunohistochemical analysis (S100, CD34, H-caldesmon, and desmin) confirmed a diagnosis of angioleiomyoma. It is noteworthy that immunohistochemistry is an important auxiliary method for differential diagnosis of angioleiomyoma from other tumors, particularly myopericytoma.

RESUMO Objetivo Verificar a incidência das lesões orais potencialmente malignas, e avaliar e correlacionar seus aspectos clínico-patológicos. Métodos A amostra foi composta pelos casos diagnosticados clinicamente como leucoplasia oral, eritroplasia oral, eritroleucoplasia, queilite actínica e líquen plano oral em um serviço de diagnóstico no período entre maio de 2012 e julho de 2013. Foram realizados testes estatísticos adotando-se nível de significância de 5% (p≤0,05). Resultados Dos 340 pacientes examinados, 106 (31,2%) se apresentaram com lesões orais potencialmente malignas; destes, 61 (17,9%) biópsias foram realizadas. A lesão mais frequente foi a queilite actínica (37,5%), e o sítio anatômico mais acometido foi o lábio inferior (49,6%). Entre os 106 pacientes da amostra, 48 (45,3%) relataram consumo de nicotina, 35 (33%) ingeriam bebidas alcoólicas e 34 (32,1%) trabalhavam expostos ao sol. Comparando o diagnóstico clínico com o histopatológico, as lesões que apresentaram maior compatibilidade foram a eritroplasia oral e a lesão ulcerada atípica (100% ambas). Conclusão Na maioria dos casos, houve compatibilidade do diagnóstico clínico com o histopatológico. Notou-se correlação entre a ocorrência de eritroplasia, leucoplasia e eritroleucoplasia com o hábito de fumar, e entre a queilite actínica com a exposição solar. A eritroleucoplasia foi a lesão que demonstrou maior grau de malignização neste estudo. Diante do exposto, recomenda-se maior atenção por parte dos cirurgiões-dentistas ao diagnóstico das lesões orais potencialmente malignas, para aplicação da melhor conduta e controle da lesão, impedindo sua transformação maligna.

ABSTRACT Objective To determine the incidence of potentially malignant oral lesions, and evaluate and correlate their clinical and pathological aspects. Methods The sample consisted of cases clinically diagnosed as oral leukoplakia, oral erythroplakia, erythroleukoplakia, actinic cheilitis, and oral lichen planus treated at a diagnostic center, between May 2012 and July 2013. Statistical tests were conducted adopting a significance level of 5% (p≤0.05). Results Out of 340 patients, 106 (31.2%) had potentially malignant oral lesions; and 61 of these (17.9%) were submitted to biopsy. Actinic cheilitis was the most frequent lesion (37.5%) and the lower lip was the most affected site (49.6%). Among 106 patients in the sample, 48 (45.3%) reported nicotine consumption, 35 (33%) reported alcohol intake and 34 (32.1%) sun exposure while working. When clinical and histopathological diagnoses were compared, oral erythroplakia and atypical ulcer were the lesions that exhibited greater compatibility (100% each). Conclusion In most cases, clinical and histopathological diagnoses were compatible. An association between the occurrence of erythroplakia, leukoplakia and erythroleukoplakia with smoking was observed. Similarly, an association between actinic cheilitis and sun exposure was noted. Erythroleukoplakia presented the highest malignancy grade in this study. Finally, dental surgeons should draw special attention to diagnosis of potentially malignant oral lesions, choose the best management, and control the lesions to avoid their malignant transformation.

Introdução: O carcinoma de células escamosas oral (CCEO) representa mais de 90% das neoplasias malignas de cabeça e pescoço. Possui prognóstico desfavorável devido à alta frequência de metástase linfonodal e invasão local. Estudos anteriores investigaram parâmetros relacionados com o comportamento biológico do CCEO e sua correlação com o desfecho clínico. Objetivo: Avaliar dados clínicos e morfológicos em casos de carcinoma de células escamosas de língua (CCEL), correlacionando esses achados com o prognóstico. Material e métodos: Cinquenta e sete espécimes de CCEL foram obtidos de pacientes submetidos à excisão cirúrgica em um hospital de referência de Natal, Brasil. Dados clínicos, a exemplo do estágio tumor-linfonodo-metástase (TNM) e do desfecho da doença (DD), foram coletados dos prontuários médicos. Secções histológicas coradas em hematoxilina e eosina foram analisadas quanto à gradação histológica de malignidade (GHM), com base no sistema de Bryne (1998). Resultados: A maioria dos pacientes (38,6%) foi diagnosticada no estágio TNM III, e 57,9% desenvolveram metástases. A remissão do tumor ocorreu em 77,2% dos casos. O parâmetro metástase apresentou associação significativa com o DD (p = 0) e o estágio TNM (p = 0,001), constituindo, dessa forma, um bom indicador de progressão da neoplasia. Não se observou correlação de GHM e estágio TNM com DD, contudo a análise estatística revelou associação significativa entre GHM e estágio TNM (p = 0,006). Conclusão: O estadiamento clínico TNM e a GHM, avaliados em conjunto, podem ser úteis para determinar o prognóstico do CCEL.

Introduction: Oral squamous cell carcinoma (OSCC), which represents more than 90% of head and neck malignant neoplasms, has a poor prognosis due to its high frequency of lymph node metastasis and local invasion. Previous studies have investigated parameters related to the biological behavior of OSCC and its correlation with disease outcome (DO). Objective: To evaluate clinical and morphological data in cases of tongue squamous cell carcinoma (TSCC), correlating these findings with prognosis. Material and methods: Fifty-seven specimens of TSCC were obtained from patients undergoing surgical excision at a referral hospital in Natal, Brazil. Clinical data, such as tumor-node-metastasis (TNM) stage and DO, were collected from medical records. Hematoxylin and eosin-stained sections were analyzed regarding histological grade of malignancy (HGM), based on the system proposed by Bryne (1998) Results: The majority of patients (38.6%) were diagnosed as TNM stage III, and 57.9% developed metastases. Remission of the tumor occurred in 77.2% of the cases. The parameter “metastasis” exhibited a significant association with DO (p = 0) and TNM stage (p = 0.001), thus constituting a good indicator of tumor progression. Correlation of HGM and TNM stage with DO was not evidenced. Nevertheless, statistical analysis showed a significant association between HGM and TNM stage (p = 0.006). Conclusion: TNM clinical staging and HGM, evaluated in association, may be useful to estimate the prognosis of TSCC.

Objetivo:Esta pesquisa objetiva estabelecer a prevalência, os fatores associados ao surgimento e as características clínicas e histopatológicas das leucoplasias e eritroplasias orais, a fim de que estes dados possam contribuir na estratégia de prevenção ao desenvolvimento dessas lesões e, consequentemente, do câncer oral.Material e método:Foi realizado estudo retrospectivo em um serviço de referência da Universidade Federal do Rio Grande do Norte (UFRN) no período de 2000 a 2012. Para verificar a associação das variáveis foi utilizado o teste qui-quadrado e o exato de Fisher (p ≤ 0,05).Resultados:Foram investigados 6.560 prontuários, tendo sido selecionados 54 casos, dos quais 44,4% do sexo masculino e 55,6% do feminino, com idade média de 56,93 anos. Com relação aos hábitos, 72,2% eram fumantes e 25% usuários de álcool, sendo que 17,9% exibiam associação dos dois hábitos. As leucoplasias orais foram as lesões mais prevalentes (0,65%), e as eritroplasias orais apresentaram maior associação com apresentação maligna no diagnóstico histopatológico (p = 0,001). A maioria das lesões não apresentou sintomatologia (p = 0,004). A leucoplasia oral foi mais frequente em mulheres fumantes com média de idade de 57 anos.Conclusão:O conhecimento dos fatores associados e das características clínicas das eritroplasias e leucoplasias orais é essencial para o estabelecimento do correto diagnóstico e tratamento.

Objective:This research aims to establish the prevalence, factors associated with the onset, and clinical and histopathological features of oral leukoplakia and erythroplakia, so that these data can contribute to the prevention of these lesions and, consequently, of oral cancer.Material and method:A retrospective study was conducted at a reference service of Universidade Federal do Rio Grande do Norte (UFRN) from 2000 to 2012. To verify the association of variables, Pearson's chi-squared test and Fisher's exact test (p ≤ 0.05) were used.Results:Among 6,560 investigated records, 54 cases were selected, of which 44.4% were male and 55.6% female, with mean age of 56.93 years. Regarding habits, 72.2% were smokers and 25% were alcohol users; 17.9% showed association of both habits. Oral leukoplakia lesions were the most prevalent (0.65%) and oral erythroplakia showed greater association with malignancy in the histopathological presentation (p = 0.001). Most lesions showed no symptoms (p = 0.004). The most frequent was oral leukoplakia in smoking women, with mean age of 57 years.Conclusion:Knowledge of associated factors and clinical characteristics of oral leukoplakia and erythroplakia is essential for establishing the correct diagnosis and treatment.

OBJETIVO: Este estudo objetivou avaliar a prevalência de alterações orais e sua associação com a doença enxerto versus hospedeiro (DEVH) em pacientes submetidos a transplante de medula óssea (TMO). METODOLOGIA: A amostra consistiu de 51 pacientes. Por meio de questionário, foram coletados dados sobre idade, sexo, doença, tipo e tempo de transplante, origem das células e ocorrência da DEVH. Foram realizados exames clínicos extra e intraoral por profissionais especializados. RESULTADOS: DEVH sistêmica foi observada em 32,5% dos pacientes transplantados alogênicos, todos apresentando manifestações orais. Também houve uma associação estatisticamente significante entre DEVH sistêmica e manifestações orais (P<0,001). CONCLUSÃO: Diante da prevalência de alterações orais relativamente alta associada à DEVH em pacientes submetidos ao TMO, o presente estudo confirma a necessidade de se considerar a odontologia no exame, diagnóstico, tratamento e prognóstico de possíveis complicações após o transplante de medula óssea.

PURPOSE: This study aimed to evaluate the prevalence of oral changes and their association with graft versus host disease (GVHD) in patients undergoing bone marrow transplantation (BMT). METHODS: The sample consisted of 51 BMT patients. A questionnaire was used to collect data on age, gender, disease, type and time of transplant, cell origin, and GVHD occurrence. The extraoral and intraoral clinical examinations were performed by specialized professionals. RESULTS: Systemic GVHD was observed in 32.5% of the allogeneic transplant patients, and all of the patients with GVHD had oral manifestations. There was a statistically significant association between systemic GVHD and oral manifestations (P<0.001). CONCLUSION: Given the relatively high prevalence of oral changes associated with GVHD in patients undergoing BMT, this study confirms the need to consider dental aspects in the examination, diagnosis, treatment and prognosis of possible complications after BMT.

OBJETIVO: relatar um caso de displasia cemento-óssea florida (DCOF) e osteomielite esclerosante difusa crônica (OEDC) em uma apresentação simultânea, enfatizando a importância do diagnóstico diferencial e adequado manejo dessas condições. DESCRIÇÃO DO CASO: Uma paciente do sexo feminino, 69 anos de idade, de cor negra, tinha uma queixa de dor e coceira na região esquerda da mandíbula. A paciente apresentava uma massa dura amarelada envolvendo todos os quadrantes dos maxilares. Foi obtida uma radiografia panorâmica, revelando lesão radiopaca lobular, difusa e irregular. Com base nos aspectos clínicos e histopatológicos, o diagnóstico foi de DCOF associada com osteomielite secundária. CONCLUSÃO: esse relato reforça a necessidade de uma avaliação precisa dos aspectos clínicos, radiográficos e histopatológicos para o diagnóstico e seleção correta de tratamento em casos de lesões combinadas.

PURPOSE: To report a case of florid cemento-osseous dysplasia (FCOD) and chronic diffuse sclerosing osteomyelitis (CDSO) in a simultaneous presentation, emphasizing the significance of differential diagnosis and appropriate management of these conditions. CASE DESCRIPTION: A female, 69 years old, black patient had a complaint of itching and pain in posterior left mandibular region. The patient had a yellowish hard mass throughout all quadrants of the jaws. A panoramic radiograph showed a lobular, diffuse and irregular radiopaque lesion. Based on clinical and histopathological findings, the diagnosis was FCOD associated with secondary osteomyelitis. CONCLUSION: This report reinforces the need of accurate assessment of clinical, radiographic and histopathological aspects for the diagnosis and correct selection of treatment in cases of combined lesions.

A síndrome de Sjögren é uma doença inflamatória sistêmica, de natureza auto-imune, caracterizada pela infiltração linfocitária progressiva de vários órgãos exócrinos e não exócrinos. Acomete preferencialmente as glândulas salivares e lacrimais determinando prejuízo estrutural e disfunção secretória destes órgãos. A produção de auto-anticorpos e a hipergamaglobulinemia policlonal indicam que anormalidades na imunidade humoral desempenham um papel importante na patogenia desta afecção e o seu diagnóstico é baseado na combinação de vários achados clínicos e laboratoriais. A proposta deste trabalho é apresentar um caso clínico de síndrome de Sjögren enfatizando a sua importância clínica e a necessidade do diagnóstico precoce na tentativa de melhorar a qualidade de vida dos pacientes acometidos.

Sjögren's syndrome is an autoimmune systemic inflammatory disease characterized by progressive lymphocytic infiltration of several exocrine and non-exocrine organs. It affects primarily the lachrymal and salivary glands causing structural damage and secretory dysfunction of the organs. The production of autoantibodies and polyclonal hypergammaglobuinemia indicate that abnormalities of humoral immunity play a significant role in this pathogenesis and its diagnosis is based upon a combination of several clinical and laboratory findings. The purpose of this report is to present one clinical case of Sjögen's syndrome emphasizing its clinical importance and necessity of the earlier diagnosis to improve the patient's quality of life.

El presente trabajo relata un inusual caso clínico de liquen plano oral (LPO) en una paciente de 44 años, que poseía varias áreas de la mucosa oral afectadas, entre ellas, encía, labio, mucosa de los carrillos y lengua, estando algunas lesiones asociadas a sintomatología dolorosa. Además, la paciente exhibía lesiones de coloración violeta con prurito en la piel, las cuales también fueron constatadas en dos de sus parientes y diagnosticadas como liquen plano. El padre y la hermana del paciente también eran afectados por lesiones similares diagnosticadas previamente como liquen plano. Dos de las regiones afectadas de la boca fueron sometidas a biopsia incisional y diagnosticadas histopatologicamente como LPO asociado a historia familiar. El tratamiento consistió en la administración de corticoide (prednisona) por vía sistémica, siendo observada remisión de las alteraciones cutáneas y una mejoría significativa de las lesiones orales después de 20 días de iniciado el dicho tratamiento. Se presenta y discute aún características específicas relacionadas con liquen plano familiar descritas en la literatura.

The aim of this paper is to report a very unusual case of oral lichen planus (OLP) in a female patient, 44 years old, which was affected in distinct sites of the oral mucosa, such as encía, lip, buccal mucosa and tongue, associated in some areas with pain. In addition, it was identified cutaneous lesions consisted of violaceous papules localized at the leg of the patient. The father and sister’s patient were also affected by similar skin lesions diagnosed previously as lichen planus. In spite of the history and clinical appearance highly indicative, biopsy of two oral lesions was indicated and the histopathologic exam confirmed the diagnosis of OLP, associated with a familial history. The treatment consisted of systemic corticosteroid (prednisone) and the patient showed significant advances after 20 days of the initial therapy. The authors also present and discuss specific features related to familial lichen planus described in the literature.