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OBJECTIVE: Fungiform papillae enlargement is a common oral manifestation of neurofibromatosis type 1 (NF1). This study aimed to objectively evaluate the size, number, and symmetry of fungiform papillae in NF1 individuals and investigate the relationship between these alterations and taste perception, salivary flow, dietary habits, and BMI. MATERIALS AND METHODS: A cross-sectional case-control study was conducted on 80 participants (40 with NF1 and 40 controls), matched by age and sex. Participants underwent quantitative and morphological evaluation of fungiform papillae, gustatory perception tests, sialometry, saliva analysis, xerostomia assessment, dietary assessments, and Body Mass Index calculations. RESULTS: The NF1 group exhibited significantly larger and more asymmetric fungiform papillae and exhibited a higher detection threshold for sweet and sour tastes, as well as hyposalivation and lower preference for healthy foods compared to the controls. No correlation was found between papillae morphology, gustatory perception tests, saliva properties, xerostomia, food preferences, or BMI in the NF1 group. CONCLUSIONS: Enlarged and asymmetric fungiform papillae, hyposalivation, heightened sensitivity to sweet and sour tastes, and reduced healthy eating habits were common in NF1. Although fungiform papillae alterations seem unrelated to taste sensitivity and food preferences, further investigation is needed to better understand the mechanisms underlying these changes.

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OBJECTIVES: This study aimed to investigate nasal septum deviation (NSD), nasal bone length (NBL), and the morphology of the middle nasal conchae (MNC) and inferior nasal conchae (INC), as well as their correlations. MATERIALS AND METHODS: The sample included 56 cone-beam computed tomography scans divided into two groups: a study group (SG; individuals with NF1; n = 28) and a control group (CG; individuals without NF1; n = 28). NSD, NBL, MNC, and INC classifications were assessed. MNC images were classified as normal, bullous, paradoxical, secondary, and accessory. INC images were classified as normal, lamellar, compact, combined, and bullous. Intra- and interobserver reliability were evaluated. RESULTS: SG had a mean NSD of 11.6° (±4.5°) compared with 9.6° (±3.2°) for the CG, showing moderate deviations with no significant difference between groups. SG had a mean NBL of 22.4 mm (±3.4 mm) compared with 22.1 mm (±3.2 mm) for the CG, with a statistically significant difference. Both groups exhibited normal, bullosa, and accessory MNC classifications. SG INC were normal, lamellar, and combined, whereas CG INC were normal and lamellar. There was a weak correlation between NSD and NBL across groups. CONCLUSION: Individuals with NF1 showed longer NBL. The weak correlation between NSD and NBL suggested multifactorial influences on these variations. These findings advance our understanding of craniofacial development in NF1 and highlight the need for further research into nasal cavity involvement in this complex genetic disorder.

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BACKGROUND: Pemphigus vulgaris (PV) is a potentially life-threatening mucocutaneous autoimmune disease that affects desmoglein-1 and desmoglein-3, leading to intraepithelial vesiculobullous lesions. In the oral mucosa, PV lesions can mimic other diseases such as mucous membrane pemphigoid, other forms of pemphigus, recurrent aphthous stomatitis, erythema multiforme, Stevens-Johnson syndrome, and virus-induced ulcers like herpes simplex virus (HSV), making diagnosis challenging. The co-occurrence of PV with Crohn's disease is rare and predominantly seen in younger patients. The therapeutic mainstay for both PV and Crohn's disease usually involves systemic corticosteroids combined with immunosuppressants and immunobiological drugs. Literature indicates that the use of these drugs, particularly TNF-alpha inhibitors, for managing autoimmune diseases like Crohn's can potentially induce other autoimmune diseases known as autoimmune-like syndromes, which include episodes of lupus-like syndrome and inflammatory neuropathies. There are few cases in the literature reporting the development of PV in individuals with CD undergoing infliximab therapy. CASE REPORT: A young female with severe Crohn's disease, treated with the TNF-alpha inhibitor infliximab, developed friable pseudomembranous oral ulcerations. Histopathological and immunofluorescence analyses confirmed these as PV. The treatment included clobetasol propionate and low-level photobiomodulation, which resulted in partial improvement. The patient later experienced severe intestinal bleeding, requiring intravenous hydrocortisone therapy, which improved both her systemic condition and oral lesions. Weeks later, new ulcerations caused by herpes virus and candidiasis were identified, leading to treatment with oral acyclovir, a 21-day regimen of oral nystatin rinse, and photodynamic therapy, ultimately healing the oral infections. To manage her condition, the gastroenterologists included methotrexate (25 mg) in her regimen to reduce the immunogenicity of infliximab and minimize corticosteroid use, as the patient was in remission for Crohn's disease, and the oral PV lesions were under control. CONCLUSION: Young patients with Crohn's disease should be referred to an oral medicine specialist for comorbidity investigation, as oral PV and opportunistic infections can arise during immunosuppressive therapy. The use of TNF-alpha inhibitors in patients treated for inflammatory bowel disease, such as Crohn's, should be carefully evaluated for potential side effects, including oral PV.

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Background: Assessment of oral epithelial dysplasia is the gold standard for investigating the risk of malignant progression. The World Health Organization (WHO) methods and the binary system have limitations. This study assess the inter- and intra-observer variability of the architectural and cytological criteria and the classification of the presence and degree of epithelial dysplasia in oral lichen planus (OLP) and oral lichenoid lesion (OLL), using both the 2017 WHO criteria and the binary system. Material and Methods: The sample consisted of 65 biopsies from lesions classified as OLP and OLL according to the American Academy of Oral and Maxillofacial Pathology (AAOMP) criteria. The histological slides were reevaluated by two oral pathologists. Results: The individual alterations with most inter-observer disagreement were atypical mitotic figures (43.1%), loss of cohesion between epithelial cells (38.5%) and drop shape rete ridges ridges (38.5%). Inter-observer agreement analysis did not show statistically significant agreement regarding the classification of epithelial dysplasia grade by WHO criteria, only regarding the binary system classification (k=0.257; p=0.035). Intra-observer agreement analysis by evaluator 1 showed that the classification of epithelial dysplasia grade according to both methods had statistically significant agreement (k=0.546; p=0.004, k=0.861; p<0.001). Considering evaluator 2, only the evaluation of the WHO system classification showed statistically significant agreement (k=0.593; p=0.010). Conclusions: The evaluation of epithelial dysplasia is subjective and focal changes and inflammatory infiltrate, characteristic of OLP and OLL, can increase the degree of disagreement among evaluators. The binary system presents better inter-observer agreement, while the WHO system presents better intra-observer agreement. Key words:Oral lichen planus, oral lichenoid lesion, oral lichenoid disease, dysplasia, inter-observer variation.

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Cheilitis is a term given to the inflammation that occurs in the vermillion of the lips. The exfoliative type is an uncommon form of cheilitis, which is characterized by inflammation and desquamation of the lip. It can cause aesthetic problems and compromise daily eating and phonation. The aim of this paper is to describe two cases of exfoliative cheilitis in young persons under periods of emotional stress and parafunctional habits. A 22-year-old white male and an 18-year-old black female presenting edema, intense dryness, and slight desquamation on the vermilion of the lips. In the second case, fissures with bleeding were also observed. Oral lesions were associated with intense emotional stress. The diagnosis of both was made based on the clinical presentation and the exclusion of other conditions. Although the patients have presented a significant improvement after the corticosteroid treatment, they still have a recurrence in stressful episodes. Detailed clinical examination and complementary exams are fundamental for determining associated factors and correctly diagnosing exfoliative cheilitis. Treatment can be challenging, especially in the face of relapses. Key words:Cheilitis, exfoliative cheilitis, oral lesions, stress psychological.

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OBJECTIVE: To evaluate the prevalence and clinical aspects of oral candidiasis in patients hospitalised in the intensive care unit. METHODS: This is a longitudinal and prospective study that included 48 participants hospitalised in the intensive care unit. Sociodemographic data, presence of systemic disorders, use of medications, laboratory tests, cause of hospital admission, type of breathing, and length of hospital stay were obtained from medical records. Oral clinical inspection and cytopathological examinations were performed on all participants. The diagnosis of clinical candidiasis was based on the presence of clinical alterations together with positive cytopathological examination results. The diagnosis of subclinical candidiasis was based on the absence of clinical lesions and a positive cytopathological examination. The absence of oral candidiasis was considered when the participant did not present oral lesions and had a negative cytopathological examination. RESULTS: Clinical candidiasis was present in 18.8% of the 48 participants, and 45.8% of them had the subclinical form. Levels of urea (P = 0.005), creatinine (P = 0.009), haemoglobin (P = 0.009), haematocrit (P = 0.011), bands (P = 0.024), international normalised ratio (INR; P = 0.034), types of breathing (P = 0.017), length of hospital stay (P = 0.037), and outcome (P = 0.014) demonstrated statistically significant differences between the groups with and without oral candidiasis. CONCLUSIONS: Clinical and subclinical forms of oral candidiasis are frequent in intensive care unit patients. Levels of urea, creatinine, haemoglobin, haematocrit, bands, INR, type of breathing, length of hospital stay, and outcome can be associated with the presence of candidiasis.

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Aim:This paper aims to report an atypical oral HSV infection diagnosed by cytopathological examination in a patient with acute lymphocytic leukemia. Case report: A nine-year-old white female was admitted with acute lymphocytic leukemia, presenting ulcers covered with pseudomembrane and spontaneous bleeding on the left soft palate, measuring approximately 2 cm, as well as other ulcers measuring 1 cm on the left lateral border of the tongue. Exfoliative cytopathology revealed neutrophils and cytopathic effects of HSV in the keratinocytes in a fibrin background. Based on the exfoliative examination, the diagnosis of HSV infection was obtained. Conclusion: Oral HSV infection can be atypical in immunocompromised patients and can cause high morbidity and mortality. Healthcare professionals, especially those working in a hospital environment, should be aware of the possibility of HSV infection in atypical lesions in these patients and evaluate the need to include antiviral prophylactic therapy.

Objetivo:Este trabalho tem como objetivo relatar uma infecção oral atípica por HSV diagnosticada por exame citopatológico em um paciente com leucemia linfocítica aguda. Relato de caso:Paciente do sexo feminino, nove anos, branca, portadora de leucemia linfocítica aguda, apresentando úlceras recobertas por pseudomembrana e sangramento espontâneo em palato mole esquerdo medindo aproximadamente 2 cm, além de outras úlceras medindo 1 cm na borda lateral esquerda da língua. A citopatologia esfoliativa revelou neutrófilos e efeitos citopáticos do HSV nos ceratinócitos em um fundo de fibrina. Com base no exame esfoliativo, foi obtido o diagnóstico de infecção por HSV. Conclusão: A infecção oral por HSV pode ser atípica em pacientes imunocomprometidos e pode causar alta morbidade e mortalidade. Os profissionais de saúde, principalmente os que atuam em ambiente hospitalar, devem estar atentos à possibilidade de infecção pelo HSV em lesões atípicas nesses pacientes e avaliar a necessidade de inclusão de terapia antiviral profilática.

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BACKGROUND: Neurofibromatosis 1 (NF1) is a common autosomal dominant syndrome with complete penetrance and highly variable expressivity. The cutaneous neurofibroma (Cnf) and plexiform neurofibroma (Pnf), café-au-lait spots, and freckle-like lesions are common in NF1, but many other manifestations can occur. We aimed to evaluate head circumference, height, weight, body mass index (BMI), head circumference-to-height ratio (HCHR) and waist-hip ratio (WHR) in adult NF1 Brazilian individuals versus a paired control group and investigate their correlation with the presence of clinically visible Pnfs, and number of "skin neurofibromas" (Snf), which include both cutaneous and subcutaneous neurofibromas. METHODS: A case-control study was conducted with 168 individuals, 84 with NF1 and 84 without NF1, paired by sex and age. Head circumference and anthropometric measurements, Snf quantification, evaluation of clinically visible Pnf and familial inheritance were accessed. RESULTS: Prevalence of macrocephaly was significantly higher in NF1 women. Height and weight were significantly lower in both males and females with NF1. HCHR was higher in the NF1 group than in the control group for both sexes. BMI was significantly lower in men with NF1. Waist and hip circumferences were significantly reduced in NF compared with the controls, but the mean WHR was significantly lower only in NF1 women. No correlation was found between the Snf and head circumference and anthropometric measurements, sex or family history. The presence and larger size of clinically visible plexiform neurofibromas were associated with normal stature (p = 0.037 and p = 0.003, respectively). CONCLUSIONS: NF1 individuals have increased prevalence of macrocephaly, short stature, low BMI, and reduced abdominal fat. There is no relation between head circumference and anthropometric data with family history, or neurofibromas.

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BACKGROUND: The diagnosis of oral lichenoid lesions (OLL) remains a challenge for clinicians and pathologists. Although, in many cases, OLL cannot be clinically and histopathologically distinguishable from oral lichen planus (OLP), one important difference between these lesions is that OLL has an identifiable etiological factor, e.g. medication, restorative material, and food allergy. The list of drugs that can cause OLL is extensive and includes anti-inflammatory drugs, anticonvulsants, antihypertensives, antivirals, antibiotics, chemotherapeutics, among others. This work aimed to perform a literature review of OLL related to chemotherapy drugs and to report two cases of possible OLL in patients with B-cell and T-cell non-Hodgkin lymphomas in use of chemotherapy and adjuvant medications. We also discuss the challenge to clinically and histopathologically differentiate OLL and OLP. CASE PRESENTATION: In both cases, oral lesions presented reticular, atrophic, erosive/ulcerated, and plaque patterns. The diagnosis of OLL was initially established in both cases by the association of histopathology and history of onset of lesions after the use of medications. Although the patients have presented a significant improvement in the oral clinical picture for more than 2 years of follow-up, they still have some lesions. CONCLUSION: A well-detailed anamnesis associated with the drug history, temporal relationship of the appearance of the lesions, and follow-up of patients are fundamental for the diagnosis of OLL related to drugs. Nevertheless, its differentiation from OLP is still a challenge.

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The most common oral choristomas are consisted of thyroid tissue and bone. The presence of sebaceous glands in the oral mucosa, especially in the buccal mucosa and labial mucosa, is often considered a normal anatomical variation since they are observed in about 80% of the population and are called ectopic sebaceous glands or Fordyce's granules. However, the presence of these glands on the tongue is rare, with only 11 cases in the dorsum of the tongue reported in the English literature, and it is considered a choristoma. This paper aims to report the third case in the literature of a congenital sebaceous choristoma on the tongue. An 11-year-old white male patient presented a firm sessile papule, without color alteration, measuring 0.4 cm x 0.3 cm in diameter, in the middle third of the dorsum of the tongue with a slight increased size in the last months. Histopathological examination showed an invagination of the epithelium into the connective tissue, forming a ductal structure covered by stratified squamous epithelium. The deeper areas had normal well-differentiated sebaceous glands, with ducts connected to the central duct. Considering clinical and histopathological findings the diagnosis was sebaceous choristoma. Despite being rare, sebaceous choristomas should also be considered in the differential diagnosis of tongue abnormalities or lesions. Although the pathogenesis is not well understood, the present report, as a congenital choristoma case in the midline, reinforces the hypothesis of a disorder with embryological origin and a possible relationship with thyroglossal duct remnants. Key words:Choristoma, Oral Mucosa, Tongue.

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BACKGROUND: Neurofibromatosis type 1 (NF1) is a chronic and progressive autosomal dominant genetic and sporadic disease characterized by cutaneous and neurological abnormalities. Plexiform neurofibroma (PN), a significant cause of clinical complications in NF-1, is a benign tumor of the peripheral nerve sheath that involves multiple nerve fascicles. Although there is an important number of patients who are affected by NF1 in Brazil, there is little data on the behavior of the disease in the national literature as well as in other low- and middle-income countries. METHODS: We performed a retrospective analysis of 491 patients with NF1 followed at two reference centers in Brazil. RESULTS: Approximately 38% of patients had PNs, resulting in reduced life quality. The median patient age with PNs was 30 years (range: 6 to 83 years). Head and neck, and extremity were the main affected locations with 35.8 and 30.6%, respectively. PNs were classified as asymptomatic in 25.1% of patients, while 52.5% presented symptomatic and inoperable tumors. The most common manifestations related to PNs were disfigurement and orthopedic involvement. Twenty patients developed neoplasms and ten (50%) presented with malignant peripheral nerve sheath tumors (MPNST). The prevalence of MPNST in our study was 2.9%. CONCLUSIONS: Patients with NF1 experience clinically significant morbidity, especially when it is associated with PN. Though there are many patients affected by NF1 in Brazil and other low- and middle-income countries, there is little data available in the corresponding literature. Our results are comparable to the previous results reported from higher-income countries and international registries.

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OBJECTIVES: The aim of this study was to evaluate the mandibular condyles of neurofibromatosis 1 (NF1) individuals without facial plexiform neurofibroma using cone beam computed tomography images. MATERIALS AND METHODS: Eighty cone beam computed tomography scans (160 mandibular condyles) were analyzed: 40 from NF1 individuals (study group) and 40 from individuals without NF1 (control group). The anteroposterior and mediolateral dimensions, height, and volume of the mandibular condyles were measured. The mandibular condyles were classified according to their morphology: healthy (absence of morphological changes), with flattening (loss of rounded contour of at least one of the surfaces), with erosion (loss of continuity of the cortical bone), with osteophyte (exophytic formation of the condyle surface), and with sclerosis (any increase in the cortical thickness in the load-bearing areas). Furthermore, the position of the mandibular condyles in relation to the joint fossa in an anteroposterior view was classified as anterior, concentric, or posterior. RESULTS: The study group had a higher anteroposterior dimension of the mandibular condyles compared with the control group (p < 0.05). There were no differences in condylar morphology and position between both groups (p > 0.05). The morphological alterations were not associated with sex or age in any group evaluated (p > 0.05). For both groups, the concentric position was the most common. For the study group, there was a significant difference in the condylar position between the sides (p < 0.05). CONCLUSIONS: NF1 individuals without facial plexiform neurofibroma present a high prevalence of condyles with a large anteroposterior dimension and asymmetric position in the joint fossa. However, no morphological and volumetric changes were observed in the mandibular condyles of them. CLINICAL RELEVANCE: The knowledge of the TMJ alterations in individuals with NF1 is important to establish an evaluation protocol, which would allow early intervention if indicated.

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Dentinogenic ghost cell tumor (DGCT) and ghost cell odontogenic carcinoma (GCOC) form a spectrum of rare benign and malignant odontogenic neoplasms, respectively. The aim of this study was to perform a comparative systematic review of the clinicopathological, genetic, therapeutic, and prognostic features of DGCT and GCOC. The electronic search was performed until December 2020 on seven electronic databases. Case reports, series, and research studies with enough histopathological criteria for diagnosis and all genomic studies were included. Both DGCT and GCOC showed a male prevalence (p = 0.043), with mandibular and maxillary predilections, respectively (p = 0.008). Peripheral DGCT (DGCTp) affected most elderly people (p < 0.001), and central DGCT (DGCTc) and GCOC occurred mainly in younger individuals. Unilateral enlargement of maxilla or mandible was the most common clinical sign associated with a radiolucent or mixed image. Ameloblastomatous epithelium was often present in both neoplasms. Basaloid and large cells with vesicular nuclei were also frequently seen in GCOC. ß-catenin expression and mutations (CTNNB1 gene) were found in DGCT and GCOC. Conservative surgery was mostly used for DGCTp, while radical resection was chosen for DGCTc and GCOC. High recurrence rates were found in DGCTc and GCOC. Metastasis occurred in 16.7% of GCOC cases and the 5-year survival rate was 72.6%. DGCT and GCOC share numerous clinicopathological features and demand a careful histopathological evaluation, considering the overlap features with other odontogenic tumors and the possibility of malignant transformation of DGCT. A strict regular post-operative follow-up is mandatory due to high recurrence rates and metastatic capacity in GCOC.

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BACKGROUND: Neurofibromatosis type 1 (NF1) is an autosomal dominant genetic disorder with a prevalence of 1:3000 births and a wide variety of clinical manifestations. Cutaneous neurofibromas (cNF) are among the most common visible manifestations of NF1 and present a major clinical burden for patients. NF1 patients with cNF often report decreased quality of life, emotional well-being and physical comfort. Developing effective medical therapies for cNF has been identified as a priority for the majority of adults with NF1. METHODS: The study was an open, controlled and prospective proof-of-concept clinical trial. The topical treatment consisted of two steps: cNF microporation using a laser device followed by topical application of one drop of diclofenac 25 mg/mL on the surface of the cNF (T neurofibroma = treatment) or physiological saline (C neurofibroma = control) and reapplied twice daily for 3 days. Neurofibroma assessments included visual and dermatoscopy observations noting color and presence of necrosis, presence of flaccidity, measurements in two dimensions, photographs, and histopathology after excision. The primary efficacy variable was the presence of tissue necrosis. The primary safety variable was the occurrence of treatment-related adverse events. RESULTS: Six patients were included in the study. The treatment resulted in transitory topical changes (healing of the microporation grid with formation of scintillating tissue layer, hyperemia and desquamation), with no statistically significant variation in the dimensions of the T and C neurofibromas in relation to pretreatment measurements. There was no necrosis in the T or C neurofibromas. In the histopathological analysis, there was no significant difference in the distribution of chronic (lymphocytic) inflammatory infiltrate in the papillary reticular dermis (subepithelial), type of infiltrate (diffuse, perivascular, or both), presence of fibrosis, and presence of atrophy among the T and C neurofibromas. No adverse events attributable to the use of diclofenac were reported during the treatment period. CONCLUSIONS: Treatment did not result in significant alterations in terms of presence of tissue necrosis, size, or histopathological features in the T neurofibromas or in comparison to the C neurofibromas. Topical diclofenac with laser microporation was well-tolerated, with no adverse events attributable to diclofenac reported. Whether these observations are due to minimal systemic and neurofibroma exposure remain to be explored in dosage studies with larger patient groups. TRIAL REGISTRATION: ClinicalTrials.gov (NCT03090971) retrospectively registered March 27, 2017.

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Objective: To investigate the efficacy and safety of deoxycholic acid (DOC) for SMF reduction.Methods: We conducted a systematic review and meta-analysis of randomized controlled trials. We searched PubMed/MEDLINE, EMBASE, and Cochrane databases until June 2020. Efficacy outcomes: Clinician-Reported Submental Fat Rating Scale; Patient-Reported Submental Fat Rating Scale; Subject Self-Rating Scale; SMF reduction measured using caliper and resonance magnetic imaging; Early therapeutic success. Safety outcomes: Withdrawals due to adverse events (AEs), Rates of AEs, Skin laxity.Results: Five studies were included, comprising 1,838 participants. DOC (1 or 2 mg/cm2) had greater improvement in all efficacy measures compared to placebo. No differences were seen between both doses of DOC. Withdrawals due to AEs were low with 1 and 2 mg/cm2 of DOC (6.8% vs. 9.9%, respectively), and there was no difference between the two doses (p = 0.22). AEs were usually associated with the injection site, were predominantly transient, and commonly resolved within the treatment session interval. Injection site pain, hematoma, anesthesia/numbness, erythema, and swelling/edema were the most common AEs. There was no difference in their prevalence between both doses of DOC.Conclusions: DOC is effective and safe for SMF reduction with no differences between doses of 1 and 2 mg/cm2.

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INTRODUCTION: Neurofibromin, a protein encoded by the NF1 gene, is mutated in neurofibromatosis 1, one of the most common genetic diseases. Oral manifestations are common and a high prevalence of hyposalivation was recently described in individuals with neurofibromatosis 1. Although neurofibromin is ubiquitously expressed, its expression levels vary depending on the tissue type and developmental stage of the organism. The role of neurofibromin in the development, morphology, and physiology of salivary glands is unknown and a detailed expression of neurofibromin in human normal salivary glands has never been investigated. AIM: To investigate the expression levels and distribution of neurofibromin in acinar and ductal cells of major and minor salivary glands of adult individuals without NF1. MATERIAL AND METHOD: Ten samples of morphologically normal major and minor salivary glands (three samples of each gland: parotid, submandibular and minor salivary; and one sample of sublingual gland) from individuals without neurofibromatosis 1 were selected to assess neurofibromin expression through immunohistochemistry. Immunoquantification was performed by a digital method. RESULTS: Neurofibromin was expressed in the cytoplasm of both serous and mucous acinar cells, as well as in ducts from all the samples of salivary glands. Staining intensity varied from mild to strong depending on the type of salivary gland and region (acini or ducts). Ducts had higher neurofibromin expression than acinar cells (p = 0.003). There was no statistical association between the expression of neurofibromin and the type of the salivary gland, considering acini (p = 0.09) or ducts (p = 0.50) of the four salivary glands (parotid, submandibular, minor salivary, and sublingual gland). Similar results were obtained comparing the acini (p = 0.35) and ducts (p = 0.50) of minor and major salivary glands. Besides, there was no correlation between the expression of neurofibromin and age (p = 0.08), and sex (p = 0.79) of the individuals, considering simultaneously the neurofibromin levels of acini and duct (n = 34). CONCLUSION: Neurofibromin is expressed in the cytoplasm of serous and mucous acinar cells, and ductal cells of salivary glands, suggesting that this protein is important for salivary gland function.

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The Ki-67 labeling index is traditionally used to investigate tumor aggressiveness. However, no diagnostic or prognostic value has been associated to the heterogeneous pattern of nuclear positivity. The aims of this study were to develop a classification for the patterns of Ki-67-positive nuclei; to search scientific evidence for the Ki-67 expression and location throughout the cell cycle; and to develop a protocol to apply the classification of patterns of Ki-67-positive nuclei in squamous epithelium with different proliferative activities. Based on empirical observation of paraffin sections submitted to immunohistochemistry for the determination of Ki-67 labeling index and literature review about Ki-67 expression, we created a classification of the patterns of nuclear positivity (NP1, NP2, NP3, NP4, and mitosis). A semi-automatic protocol was developed to identify and quantify the Ki-67 immunostaining patterns in target tissues. Two observers evaluated 7000 nuclei twice to test the intraobserver reliability, and six evaluated 1000 nuclei to the interobserver evaluation. The results showed that the immunohistochemical patterns of Ki-67 are similar in the tumoral and non-tumoral epithelium and were classified without difficulty. There was a high intraobserver reliability (Spearman correlation coefficient > 0.9) and moderate interobserver agreement (k = 0.523). Statistical analysis showed that non-malignant epithelial specimens presented a higher number of NP1 (geographic tongue = 83.8 ± 21.8; no lesion = 107.6 ± 52.7; and mild dysplasia = 86.6 ± 25.8) when compared to carcinoma in Situ (46.8 ± 34.8) and invasive carcinoma (72.6 ± 37.9). The statistical evaluation showed significant difference (p < 0.05). Thus, we propose a new way to evaluate Ki-67, where the pattern of its expression may be associated with the dynamics of the cell cycle. Future proof of this association will validate the use of the classification for its possible impact on cancer prognosis and guidance on personalized therapy.

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ABSTRACT: Lichen planus (LP) is a mucocutaneous immune-mediated disease of unknown etiology. It is more prevalent in women and usually occurs between the third and sixth decades of life. Oral lesions may or may not be associated with skin and genital lesions. Although the role of genetic factors is still undetermined, reports of LP in more than one family member are not uncommon. However, the occurrence of LP in monozygotic twins is rare. We report a rare case of 42-year-old female monozygotic twins presenting oral LP. This report is even rarer because one of the patients had cutaneous lesions of an unusual variant of LP (LP pigmentosus) and the other had an uncommon association with lichen sclerosus. The etiology and pathogenesis of LP are still uncertain. However, despite being rare, its occurrence in family members and monozygotic twins suggests that genetic factors are involved in its development.

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