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Highest Dose of Eslicarbazepine for Refractory Secondary Trigeminal Neuralgia
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
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
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
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
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
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
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
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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