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    Highest Dose of Eslicarbazepine for Refractory Secondary Trigeminal Neuralgia

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    This is the first case reporting on ESL used at its highest dosage of 1600mg in a case of TN secondary to M

    oral pemphigus

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    Pemphigus is a group of potentially life-threatening autoimmune diseases characterized by cutaneous and/or mucosal blistering, due to the presence circulating IgG antibodies directed against desmoglein 1 and 3 (Dsg 1 and 3). Differences in the particular distribution of these result in different clinical manifestations of the disease. The most common variant is pemphigus vulgaris (PV). There is a fairly strong genetic background to pemphigus with linkage to HLA class II alleles and ethnic groups such as Ashkenazi Jews and those of Mediterra-nean and Indian origin are especially liable. Oral lesions are commonly characterized by the presence of vesiculobullous and ulcerative lesions. Diagnosis is achieved via three different parameters: perilesional tissue biopsy, histological and immunological examinations. Serum autoantibodies to either Dsg1 or Dsg3 are best detected using both normal human skin and monkey esophagus or by enzyme-linked immunosorbent assay. The main aim of treatment is to reduce inflammatory re-sponse and autoantibody production, in order to achieve disease remission in a short time. Before the advent of corticosteroids, PV was typically fatal due to dehydration or secondary systemic infections. Current treatment is largely based on systemic immunosuppression using corticosteroids, with azathioprine or other adjuvants or alternatives. Nonetheless, newer therapies, such as intravenous immunoglobulins (IVIg) or anti-CD20 monoclonal antibodies (Rituximab), with potentially fewer adverse effects also appear promisin

    Escalating dose of adalimumab as monotherapy to treat unusual giant and refractory oral-pharyngeal ulcerations in Crohn's disease

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    Crohn's disease (CD) is a chronic, autoimmune inflammatory disease which may affect the entire gastrointestinal tract, from the oral cavity to the anus. Oral-pharyngeal ulcerations may be significant and persistent in patients with established CD, and the use of TNF-α inhibitor has demonstrated to be useful. We report a unique case of an unusual manifestation of oral CD characterized only by multiple, giant, long-lasting, relapsing ulcerations successfully treated with an escalating dose of Adalimumab at 40 mg weekly as a monotherapy

    Gastric diffuse large B-cell lymphoma (DLBCL) exhibiting oral acanthosis nigricans and tripe palms

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    Approximately one-third of non-Hodgkin lymphomas arise primarily from sites other than lymph nodes, such as spleen or bone marrow, and even from sites which normally contain no native lymphoid tissue. The extranodal lymphomas represent a challenge in routine lymphoma diagnosis, due to the variety of histological types, molecular abnormalities and clinical pictures [1]. Diffuse large B-cell lymphoma (DLBCL) is the most common extranodal lymphoma encountered in the gastrointestinal tract. This is mainly a disease of older adults in the seventh decade, even though it may occasionally affect children and young adults. It typically produces large, destructive lesions that may invade adjacent structures [2]. A 74-year-old woman was referred to our Oral Medicine Unit by the nearby Cardio-pulmonary Unit, where she was hospitalised because of severe pneumonia refractory to the antibiotic therapy. Here, routine haematological blood tests revealed lymphocytosis, mild anemia, and a slightly elevated lactate dehydrogenase, whereas a basic workup for underlying malignancy was normal, except for a mild increase of beta2-microglubulin. Clinically, diffuse micropapillary lesions on the hard palate and inner upper lips (Fig. 1A), a “cerebriform” aspect of the right cheek (Fig. 1B), and a velvety rugose appearance on palms and palmar surface of the fingers on both hands (tripe palms) (Fig. 1C and D) were detected. Oral biopsy revealed amarked epithelial thickening with papillary hyperplasia, acanthosis, and slight dyskeratosis. The dermal papillae project upward as finger-like projections with a chronic lymphomonocytic inflammatory infiltrate (Fig. 1E). Oral acanthosis nigricans (AN) associated with tripe palms was suspected. Benign AN was excluded, because no associated syndrome, obesity, or medical history of taking medicine was found. The glycosylated haemoglobin level and insulin resistant testswere normal. Conversely, malignant AN was confirmed by the presence of a hyperplastic nodular mass, with a superficial erosion, on the wall of the stomach. A biopsy revealed features consistent with DLBCL, showing a lymphoid infiltrate in the lamina propria with a predominance of small to medium-sized and scattered large cells (Fig. 1F), which turned out to be CD 20 positive (Fig. 1G), invaded and destroyed the glandular epithelium that was pancytokeratin positive (Fig. 1H). The stagewas EI2, according to theAnnArbor classificationmodified by Musshoff [3], since a total body CT scan did not detect any enlarged lymph nodes. The CT scan and tumour markers were re-performed 1 month later, some days before her demise, with negative results, as sometimes the primary tumour might not be detected for a long time [4]. Thus, no other malignancy was detected, reinforcing the association between gastric DLBCL and malignant AN. The patient died due to severe cardio-pulmonary complications. AN is a rare mucocutaneous disorder, which is characterised by cutaneous and oral papillary lesions [5]. It includes a benign and a malignant form, which is associated with an underlying, often aggressive, malignancy, either non-haematological [5] or haematological [6]. The pathogenesis of malignant AN is still unclear. A possible mechanism might involve the production of the transforming growth factor alpha (TGF-), which is closely related to the epidermal growth factor (EGF) and binds to the same receptor, EGFR. This binding activates the classical mitogen-activated protein kinase (MAPK, ERK) pathway, involved in regulating basic cellular functions such as proliferation, differentiation, and migration [7]. Since some type of cancers produce large amounts of TGF-, it is likely that keratinocyte growth might be stimulated via an endocrine route.Whether this pathogenetic mechanism might also be applied to the DLBCL remains questionable and, thus, further investigations are required

    Evaluation of Metabolomics as Diagnostic Targets in Oral Squamous Cell Carcinoma: A Systematic Review

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    In recent years, high-throughput technologies have facilitated the widespread use of metabolomics to identify biomarkers and targets for oral squamous cell carcinoma (OSCC). As a result, the primary goal of this systematic review is to identify and evaluate metabolite biomarkers and their pathways for OSCC that featured consistently across studies despite methodological variations. Six electronic databases (Medline, Cochrane, Web of Science, CINAHL, ProQuest, and Embase) were reviewed for the longitudinal studies involving OSCC patients and metabolic marker analysis (in accordance with PRISMA 2020). The studies included ranged from the inception of metabolomics in OSCC (i.e., 1 January 2007) to 30 April 2023. The included studies were then assessed for their quality using the modified version of NIH quality assessment tool and QUADOMICS. Thirteen studies were included after screening 2285 studies. The majority of the studies were from South Asian regions, and metabolites were most frequently derived from saliva. Amino acids accounted for more than quarter of the detected metabolites, with glutamate and methionine being the most prominent. The top dysregulated metabolites indicated dysregulation of six significantly enriched pathways including aminoacyl-tRNA biosynthesis, glutathione metabolism and arginine biosynthesis with the false discovery rate (FDR) <0.05. Finally, this review highlights the potential of metabolomics for early diagnosis and therapeutic targeting of OSCC. However, larger studies and standardized protocols are needed to validate these findings and make them a clinical reality

    Drug-induced oral lichenoid reactions: a real clinical entity? A systematic review

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    Drug-induced oral lichenoid reactions (DIOLRs) have been extensively reported in the literature, but the validity of the causality relationship between any drug and the oral lichenoid lesions (OLLs) still remains questionable. We sought to determine whether this causality relationship really exists, whether a resolution of the oral lesions upon withdrawal occurs, and what the most common alleged offending medications are

    Genotype–oropharyngeal phenotype correlation in Mexican patients with dystrophic epidermolysis bullosa

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    Previous investigations have attempted to correlate the genotype with the cutaneous phenotype in patients with epidermolysis bullosa (EB), but never with the oropharyngeal phenotype. Seventeen dystrophic EB (DEB) patients were genotyped for COL7A1 gene mutations and divided into five distinct groups. Oropharyngeal disease severity was assessed with the Epidermolysis Bullosa Oropharyngeal Severity (EBOS) score by an oral medicine specialist. The genotype–phenotype correlation was calculated by Kruskal–Wallis analysis of variance using the Mann–Whitney test, applying the Bonferroni correction. The most severe oropharyngeal phenotype was found in the group with the 2470insG/ 3948insT mutation, with a mean disease severity score of 18.50 2.12; the mildest was found in the 6862del16 mutation group, with a mean disease severity score of 0.57 1.13. The most significant difference in median score was found in the total score (P = 0.009), followed by tongue (P = 0.02) and upper lip (P = 0.021), but no correlation was found between disease severity and the groups (P > 0.005, after Bonferroni correction). Multiple comparisons among the five different genotypic groups revealed no statistically significant genotype–oropharyngeal phenotype correlation; it was not possible to establish which group was more severe, or to associate a specific mutation to a specific oropharyngeal phenotype

    Going Beyond Counting First Authors in Author Co-citation Analysis

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    The present study examines one of the fundamental aspects of author co-citation analysis (ACA) - the way co-citation counts are defined. Co-citation counting provides the data on which all subsequent statistical analyses and mappings are based, and we compare ACA results based on two different types of co-citation counting - the traditional type that only counts the first one among a cited work's authors on the one hand and a non-traditional type that takes into account the first 5 authors of a cited work on the other hand. Results indicate that the picture produced through this non-traditional author co-citation counting contains more coherent author groups and is therefore considerably clearer. However, this picture represents fewer specialties in the research field being studied than that produced through the traditional first-author co-citation counting when the same number of top-ranked authors is selected and analyzed. Reasons for these effects are discussed
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