Pemphigus vulgaris and mucous membrane pemphigoid: A systematic review of clinical manifestations, diagnosis, and treatment.

Pemphigus vulgaris (PV) and mucous membrane pemphigoid (MMP) are mucocutaneous autoimmune diseases characterized by blistering lesions of mucous membranes and skin, with very similar clinical manifestations. This study aimed to systematically review the literature on the clinical and demographic profile, diagnostic methods, and treatment of patients with pemphigus vulgaris (PV) and mucous membrane pemphigoid (MMP). Studies describing cases of PV and MMP diagnosed by direct immunofluorescence that exhibited intraoral manifestations were included. Thirty-two articles were included, with 18 studies on PV and 15 on MMP, corresponding to 50 and 123 cases diagnosed as PV and MMP, respectively. Most patients with PV (64 %) and MMP (81.3 %) were women in the fifth and sixth decade of life, respectively. The mouth was the primary site of involvement both in PV (71.4 %) and in MMP (91 %). The cheek mucosa and gingiva were the most frequently affected intraoral sites in PV (30 %) and MMP (64.2 %), respectively. Direct immunofluorescence was positive for IgG in all cases of the two conditions. The treatment of choice was systemic corticosteroid therapy for patients with PV (50 %) and topical treatment for patients with MMP (53.7 %). Differences in intraoral site predilection, extraoral involvement, and the results of diagnostic tests allow us to trace the clinical, demographic, and diagnostic profile of PV and MMP that contributes to differential diagnosis and therapeutic management.

Clinical and radiographic characteristics of pycnodysostosis: A systematic review.

Purpose: Pycnodysostosis (PYCD), an autosomal recessive syndrome, is characterized by an imbalance in bone remodeling that produces various clinical and radiographic craniofacial manifestations. This review represents a systematic examination of these manifestations, as well as oral features associated with PYCD. Materials and Methods: A systematic review was conducted across 8 databases from February to March 2023. The search strategy focused on studies reporting cases of PYCD that examined the clinical and radiographic craniofacial and oral characteristics associated with this syndrome. Results: The review included 84 studies, encompassing a total of 179 cases of PYCD. More than half of the patients were female (55.3%), and the mean age was 14.7 years. Parental consanguinity was reported in 51.4% of the cases. The most common craniofacial clinical manifestation was a prominent nose, observed in 57.5% of cases. Radiographically, the most frequently reported craniofacial characteristics included the presence of an obtuse mandibular angle (84.3%) and frontal cranial bosses (82.1%). Clinical and radiographic examinations revealed oral alterations, with micrognathia present in 62.6% of patients and malocclusion in 59.2%. Among dental anomalies, tooth agenesis was the most commonly reported, affecting 15.6% of patients. Conclusion: Understanding the clinical and radiographic craniofacial features of PYCD is crucial for dental professionals. This knowledge enables these clinicians to devise effective treatment plans and improve patient quality of life.

Does YAP influence cell proliferation and apoptosis in benign epithelial odontogenic lesions?

OBJECTIVE: To analyze the immunohistochemical expression of YAP and its correlation with markers involved in cell proliferation and apoptosis in benign epithelial odontogenic lesions. STUDY DESIGN: The sample consisted of 95 cases of odontogenic lesions (25 dentigerous cysts, 30 non-syndromic odontogenic keratocysts, 30 conventional ameloblastomas, and 10 unicystic ameloblastomas) and 10 dental follicles used as normal odontogenic tissue. The histological sections were submitted to immunohistochemistry with YAP, cyclin D1, Ki-67, and Bcl-2 antibodies. Immunoexpression was analyzed qualitatively and quantitatively using an adapted method. The collected data were analyzed descriptively and statistically (p ≤ 0.05). RESULTS: The highest YAP expression was observed in odontogenic keratocysts, followed by unicystic ameloblastomas and conventional ameloblastomas, which exhibited moderate immunoreactivity predominantly in peripheral cells. Furthermore, significant differences in YAP immunoexpression were observed between the groups analyzed, with significant positive correlations between YAP and cyclin D1 in dentigerous cysts and unicystic ameloblastomas and between YAP and Ki-67 in unicystic ameloblastomas (p < 0.05). However, there were no statistically significant correlations between YAP and Bcl-2 immunoexpression in the groups studied. CONCLUSION: YAP may influence epithelial cell proliferation in odontogenic cysts and tumors, suggesting its possible participation in the progression of the odontogenic lesions studied.

A 14-year retrospective study focusing on clinical and morphological features of oral cavity lipomas: A review of main topics.

BACKGROUND: Lipomas are benign soft tissue neoplasms frequently found in the human body. Head and neck lipomas are relatively uncommon, accounting for 1 to 4% of benign lesions at this location. This 14-year retrospective study analyzed the clinical-pathological features of cases of oral cavity lipomas and their histopathological variants seen at a single oral pathology referral center. MATERIAL AND METHODS: Data on age, sex, anatomical location, clinical diagnosis, and histological subtypes were collected from all cases microscopically diagnosed as lipoma. Three previously trained oral pathologists re-evaluated hematoxylin/eosin-stained slides of all selected cases. RESULTS: Among 7,861 oral and maxillofacial lesions diagnosed at the service, 95 (1.2%) were lipomas or their histopathological variants. There was a predominance of female patients (n = 65; 68%); the mean age at diagnosis was 58.8 years (±13.56). We found the following histological subtypes: conventional lipoma, fibrolipoma, spindle cell lipoma, sialolipoma, osteolipoma, chondrolipoma, and intramuscular lipoma. The buccal mucosa was the most affected site. Conventional lipoma and fibrolipoma were the most commonly diagnosed histological variants. Although most lipomas are asymptomatic, large lipomas can occur, reaching a diameter of 4 cm. CONCLUSION: The present study reinforces the importance of careful clinical and histopathological examination in order to obtain an accurate diagnosis and to ensure appropriate treatment.

Immunohistochemical study of the plasminogen activator system in benign epithelial odontogenic lesions.

The aim of this study was to analyze and compare the immunohistochemical expression of plasminogen activator system (PAS) proteins (uPA, uPAR, and PAI-1) in ameloblastomas (AMBs), odontogenic keratocysts (OKCs), and dental follicles (DFs) representing normal odontogenic tissue, as well as to investigate possible correlations between these proteins. Twenty AMBs, 20 OKCs, and 10 DFs were selected for immunohistochemical analysis. In each case, the immunoexpression of uPA, uPAR, and PAI-1 was evaluated semiquantitatively based on the percentage of positivity in odontogenic epithelial and connective tissue cells. The epithelial immunoexpression of uPA was significantly lower in AMBs when compared to OKCs (p = 0.001) and DFs (p = 0.029). Significantly higher epithelial immunostaining for uPAR was observed in AMBs when compared to OKCs (p < 0.001). There were no significant differences in the epithelial immunoexpression of PAI-1 between AMBs and OKCs (p = 1.000). The correlations found for the expression of the studied proteins were not statistically significant (p > 0.05). However, the epithelial and connective tissue expressions of uPAR have a strong positive and statistically significant correlation in AMBs. The present results suggest that uPA is involved in the pathogenesis of OKCs and that uPAR may participate in tumorigenesis in AMBs. The high percentage of PAI-1-positive cells suggests a possible role for this protein in the development of AMBs and OKCs. Furthermore, the studied proteins do not seem to act synergistically in AMBs, OKCs, and DFs.

Immunohistochemical study of the plasminogen activator system in benign epithelial odontogenic lesions

Abstract: The aim of this study was to analyze and compare the immunohistochemical expression of plasminogen activator system (PAS) proteins (uPA, uPAR, and PAI-1) in ameloblastomas (AMBs), odontogenic keratocysts (OKCs), and dental follicles (DFs) representing normal odontogenic tissue, as well as to investigate possible correlations between these proteins. Twenty AMBs, 20 OKCs, and 10 DFs were selected for immunohistochemical analysis. In each case, the immunoexpression of uPA, uPAR, and PAI-1 was evaluated semiquantitatively based on the percentage of positivity in odontogenic epithelial and connective tissue cells. The epithelial immunoexpression of uPA was significantly lower in AMBs when compared to OKCs (p = 0.001) and DFs (p = 0.029). Significantly higher epithelial immunostaining for uPAR was observed in AMBs when compared to OKCs (p < 0.001). There were no significant differences in the epithelial immunoexpression of PAI-1 between AMBs and OKCs (p = 1.000). The correlations found for the expression of the studied proteins were not statistically significant (p > 0.05). However, the epithelial and connective tissue expressions of uPAR have a strong positive and statistically significant correlation in AMBs. The present results suggest that uPA is involved in the pathogenesis of OKCs and that uPAR may participate in tumorigenesis in AMBs. The high percentage of PAI-1-positive cells suggests a possible role for this protein in the development of AMBs and OKCs. Furthermore, the studied proteins do not seem to act synergistically in AMBs, OKCs, and DFs.

Caliber Persistent Artery in the Upper Lip: A Case Report with Unusual Histopathological Findings.

Caliber persistent labial artery (CPLA) consists in a dilated portion of the main branch of the labial artery without loss of size. The aim of this study is to report a case of a patient diagnosed with CPLA in the upper lip, emphasizing unusual histopathological and immunohistochemical findings. A 67-year-old female patient with complaint of a pulsating upper lip lesion without painful symptomatology. Under a clinical diagnosis of CPLA, and considering that the patient was edentulous and used a total prosthesis, an excisional biopsy of the lesion was performed to avoid future traumas in the region and consequently possible exuberant local bleeding. At anatomopathological examination structures suggestive of lymphoid follicles and germinal centers were visualized. Immunohistochemistry showed positivity for CD20, CD68, desmin and CD34 and negativity for CD4. The patient did not have a history of allergies, cardiovascular, rheumatic or systemic diseases that could justified the findings. The case presents unusual histopathological structures, evidencing the necessity of more studies about this pathology so scarce in the literature.

Caliber persistent artery in the upper lip: a case report with unusual histopathological findings

Abstract Caliber persistent labial artery (CPLA) consists in a dilated portion of the main branch of the labial artery without loss of size. The aim of this study is to report a case of a patient diagnosed with CPLA in the upper lip, emphasizing unusual histopathological and immunohistochemical findings. A 67-year-old female patient with complaint of a pulsating upper lip lesion without painful symptomatology. Under a clinical diagnosis of CPLA, and considering that the patient was edentulous and used a total prosthesis, an excisional biopsy of the lesion was performed to avoid future traumas in the region and consequently possible exuberant local bleeding. At anatomopathological examination structures suggestive of lymphoid follicles and germinal centers were visualized. Immunohistochemistry showed positivity for CD20, CD68, desmin and CD34 and negativity for CD4. The patient did not have a history of allergies, cardiovascular, rheumatic or systemic diseases that could justified the findings. The case presents unusual histopathological structures, evidencing the necessity of more studies about this pathology so scarce in the literature.

Resumo Artéria labial de calibre persistente (ALCP) consiste em uma parte dilatada do ramo principal da artéria labial que penetra no tecido submucoso sem perda de calibre. O objetivo desse estudo é relatar um caso de uma paciente diagnosticada com ALCP em lábio superior, enfatizando os achados histopatológicos e imuno-histoquímicos incomuns. Paciente de 67 anos, sexo feminino, com queixa de lesão em lábio superior, pulsante, sem sintomatologia dolorosa. Diante do diagnóstico clínico de ALCP, e considerando que a paciente era edêntula e usuária de prótese total, foi realizada biópsia excisional para evitar futuros traumas na região e, consequentemente, sangramento local exuberante. Ao exame anatomopatológico foram visualizadas estruturas sugestivas de folículos linfoides e com formações sugestivas de centros germinativos. No exame imuno-histoquímico observou-se imunopositividade para CD20, CD68, desmina e CD34 e sem imunomarcação para CD4. A paciente relatou não possuir histórico de alergias, doenças cardiovasculares, reumáticas ou sistêmicas que justificassem os achados. O caso apresenta estruturas histopatológicas incomuns, corroborando a necessidade de mais estudos acerca dessa lesão tão pouco discutida na literatura.