Benign Mucosal Membrane Pemphigoid as a Differential Diagnosis of Necrotizing Periodontal Disease.
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ABSTRACT:
SUBMITTER: Storrer CLM
PROVIDER: S-EPMC7772047 | biostudies-literature | 2020
REPOSITORIES: biostudies-literature
Publications
Benign Mucosal Membrane Pemphigoid as a Differential Diagnosis of Necrotizing Periodontal Disease.
Storrer Carmen Lucia Mueller CLM Rigo Junior Darlan D Teixeira Lucimari L Costa Eugênio Esteves EE Endres Brayan Ruppenthal BR Dos Santos Diogo Vinícius Ferreira DVF Sebastiani Aline Monise AM
Case reports in dentistry 20201222
Desquamative gingivitis is an oral sign of systemic changes that may be symptomatic or asymptomatic. It is generally related to immunological changes. This report is aimed at describing the case of a 51-year-old female patient, who presented with clinical desquamative gingivitis and was initially diagnosed and treated as necrotizing periodontal disease, but no improvement. The cause was hypothesized to be an autoimmune disease. Drug therapy was initiated as a combination of prednisone and topica ...[more]
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Project description:Bullous pemphigoid (BP) and mucous membrane pemphigoid (MMP) sometimes have overlapping clinical, histopathological, and direct immunofluorescence (DIF) features in the early stages. Complement deposition is an intrinsic component of the patho-mechanism of BP in contrast to MMP. Hence immunohistochemistry (IHC) for C3d and C4d may be helpful in differentiating the two disorders. Seventy-four patients of BP and 18 patients of MMP along with 10 negative controls were enrolled in this study. C3d and C4d IHC was performed in formalin-fixed skin biopsy specimens. C3d IHC staining in BP/MMP had a sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) of 59.2%/41.2%, 100%/100%, 100%/100%, 25.6%/50.0%, respectively. C4d IHC staining in BP/MMP had a sensitivity, specificity, PPV and NPV of 26.8%/17.6%, 100%/100%, 100%/100% and 16.1%/41.7%, respectively. Receiver operator analysis showed utility of C3d in diagnosing both BP [Area under curve (AUC) = 0.8, p = 0.0001] and MMP (AUC = 0.71; p = 0.001). C4d was useful in diagnosis of BP (AUC = 0.5; p = 0.0001), but not MMP (AUC = 0.6; p = 0.064). Hence, C3d is a better diagnostic modality for BP as compared to C4d, whereas C3d and C4d have lower diagnostic importance in MMP. C3d IHC can be employed in diagnosing BP when a second biopsy for direct immunofluorescence (DIF) is not possible or where a facility for IF microscopy does not exist.
| S-EPMC10338454 | biostudies-literature
Project description:BackgroundMucous membrane pemphigoid is a group of chronic subepithelial autoimmune blistering diseases that mainly affect mucous membranes. Laminin 332-specific autoantibodies are present in approximately 1/3 of the patients, being associated with an increased risk of malignancy. Because of the severe complications, an early recognition of the disease allowing a timely therapy is essential. The gold standard methods for detection of laminin 332-specific autoantibodies, including the immunoprecipitation and immunoblotting are non-quantitative, laborious and restricted to a few specialized laboratories worldwide. In addition, the use of radioimmunoassays, although highly sensitive and specific, are laborious, expensive and tightly regulated. Therefore, there is a stringent need for a quantitative immunoassay for the routine detection of laminin 332-specific autoantibodies more broadly available to diagnostic laboratories. The aim of this study was to compare different antigenic substrates, including native, recombinant laminin 332 and laminin 332-rich keratinocyte extracellular matrix, for development of an ELISA to detect autoantibodies in mucous membrane pemphigoid.ResultsUsing a relatively large number of sera from MMP patients with well-characterized autoantibody reactivity we show the suitability of ELISA systems using laminin 332 preparations as adjunct diagnostic tools in MMP. While glycosylation of laminin 332 does not appear to influence its recognition by MMP autoantibodies, ELISA systems using both purified, native and recombinant laminin 332 demonstrated a high sensitivity and good correlation with the detection of autoantibodies by immunoblotting. ELISA systems using different laminin 332 preparations represent a feasible and more accessible alternative for a broad range of laboratories.ConclusionsOur findings qualify the use of immunoassays with the laminin 332-rich preparations as an ancillary diagnostic tool in mucous membrane pemphigoid.
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Project description:PurposeTo evaluate preferred diagnostic tools and treatment decision-making factors in cases suspicious of mucous membrane pemphigoid (MMP) amongst ophthalmologists and cornea specialists.MethodsWeb-based survey, consisting of 14 multiple choice questions, posted to the Cornea Society Listserv Keranet, the Canadian Ophthalmological Society Cornea Listserv, and the Bowman Club Listserv.ResultsOne hundred and thirty-eight ophthalmologists participated in the survey. Eighty-six percent (86%) of respondents were cornea trained and practiced in either North America or Europe (83%). Most respondents (72%) routinely perform conjunctival biopsies for all suspicious cases of MMP. For those who do not, fear that biopsy will exacerbate inflammation was the most common reason to defer investigation (47%). Seventy-one percent (71%) performed biopsies from perilesional sites. Ninety-seven percent (97%) ask for direct (DIF) studies and 60% for histopathology in formalin. Most do not recommend biopsy at other non-ocular sites (75%), nor do they perform indirect immunofluorescence for serum autoantibodies (68%). Immune-modulatory therapy is started following positive biopsy results for most (66%), albeit most (62%) would not let a negative DIF influence the choice of starting treatment should there be clinical suspicion of MMP. Differences in practice patterns as they relate to level of experience and geographical location are contrasted to the most up-to-date available guidelines.ConclusionResponses to the survey suggest that there is heterogeneity in certain practice patterns for MMP. Biopsy remains an area of controversy in dictating treatment plans. Identified areas of need should be targeted in future research.
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Project description:Intravenous immunoglobulin (IVIg) is used to treat mucous membrane pemphigoid (MMP), although its therapeutic effectivity is not sufficiently supported by randomized controlled clinical trials and its mode of action is only insufficiently understood. We have examined the effect of IVIg in a mouse model of anti-laminin 332 MMP and found that IVIg ameliorates both cutaneous and mucosal inflammatory lesions. Our investigation into the modes of action of IVIg in MMP indicated effective antiinflammatory mechanisms beyond the enhanced degradation of IgG mediated through inhibition of the neonatal Fc receptor. Our results suggest that IVIg curbs the activation of neutrophils at several levels. This includes a direct, immediate inhibitory effect on neutrophil activation by immune complexes but not C5a which blunts the release of reactive oxygen species and leukotriene B4 from neutrophils. IVIg also suppresses the formation of neutrophil extracellular traps in response to Ca2+ ionophore. In vivo treatment with IVIg altered the transcriptome of blood leukocytes and bone marrow neutrophils towards less proinflammatory phenotypes. Collectively, our results support the effectivity of IVIg in the treatment of MMP and indicate that effects on neutrophils at multiple levels may significantly contribute to its therapeutic effects.
2024-04-10 | GSE263026 | GEO
Project description:PurposeTo investigate the value of spectral CT in the differential diagnosis of benign from malignant pleural effusion.Method and materials14 patients with benign pleural effusion and 15 patients with malignant pleural effusion underwent non-contrast spectral CT imaging. These patients were later verified by the combination of disease history, clinical signs and other information with the consensus of surgeons and radiologists. Various Spectral CT image parameters measured for the effusion were as follows: CT numbers of the polychromatic 140kVp images, monochromatic images at 40keV and 100keV, the material density contents from the water, fat and blood-based material decomposition images, the effective atomic number and the spectral curve slope. These values were statistically compared with t test and logistic regression analysis between benign and malignant pleural effusion.ResultsThe CT value of benign and malignant pleural effusion in the polychromatic 140kVp images showed no differences (12.61±3.39HU vs. 14.71±5.03HU) (P>0.05), however, they were statistically different on the monochromatic images at 40keV (43.15±3.79 vs. 39.42±2.60, p = 0.005) and 100keV (9.11±1.38 vs. 6.52±2.04, p<0.001). There was difference in the effective atomic number value between the benign (7.87±0.08) and malignant pleural effusion (7.90±0.02) (P = 0.02). Using 6.32HU as the threshold for CT value measurement at 100keV, one could obtain sensitivity of 100% and specificity of 66.7% with area-under-curve of 0.843 for differentiating benign from malignant effusion. In addition, age and disease history were potential confounding factors for differentiating malignant pleural effusion from benign, since the older age (61.13±12.51 year-old vs48.57±12.33 year-old) as well as longer disease history (70.00±49.28 day vs.28.36±21.64 day) were more easily to be found in the malignant pleural effusion group than those in the benign pleural effusion group. By combining above five factors, one could obtain sensitivity of 100% and specificity of 71.4% with area-under-curve of 0.933 for differentiating benign from malignant effusion.ConclusionThe CT value measurement at both high and low energy levels and the effective atomic number obtained in a single spectral CT scan can assist the differential diagnosis of benign from malignant pleural effusion.Combining them with patient age and disease history can further improve diagnostic performance.Clinical relevance/applicationClinical findings and Spectral CT imaging can provide significant evidences about the nature of pleural effusion.
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Project description:PurposeOcular mucous membrane pemphigoid (OcMMP) is a rare eye disease characterized by relapsing-remitting or persisting long-lasting inflammatory events associated with progressive scarring. Despite long-term immunomodulating therapy, abnormal fibrosis keeps worsening in patients with OcMMP. This study investigates the fibrotic process in patients with OcMMP, as well as the critical role of the epithelium in modulating the local fibrosis.MethodsIn this prospective, observational pilot study, patients affected by long-lasting OcMMP were compared with age- and gender-matched healthy controls. Clinical grading was assessed, and conjunctival biopsy and impression cytology were performed. Conjunctival samples were used for quantifying the expression of transcripts regulating the inflammatory and fibrogenic processes.ResultsOcular surface clinical and functional markers worsened in patients with OcMMP with fibrotic disease progression. In more advanced disease stages, both impression cytologies and conjunctival biopsies revealed increased tissue remodeling and profibrotic markers (α-SMA and TGF-β), and decreased levels of inflammatory markers (I-CAM1, IL-10, and IL-17). Increased epithelial expression of profibrotic markers and histological changes were detected.ConclusionsChronic OcMMP is characterized by a progressive, aberrant self-sustaining fibrotic process that worsens clinical signs and symptoms. Conjunctival epithelial cells may transdifferentiate into myofibroblast-like phenotypes when chronically exposed to high levels of inflammation, as in the case of OcMMP. Tissue remodeling markers in OcMMP could be used as early diagnostic, prognostic, and therapeutic biomarkers, harvested in a non-invasive and painless procedure such as impression cytologies.
| S-EPMC10723221 | biostudies-literature
Project description:Background and aimsIn the case of mucous membrane pemphigoid with gingival expression (gMMP), the complete healing of the gingiva is generally not achieved despite medical treatment. Therefore, patients' oral comfort is impaired. The dysbiotic periodontal microbiota, generated by a lack of oral hygiene associated with persistent gingival pain, could the immunopathological mechanism to persist. The main objective of this study was to characterize the subgingival microbiota of the gMMP patients, and to highlight a potential link between this microbiological data and the clinical data.MethodsSubgingival biofilm was collected from 15 gMMP patients, medically treated or not, but not receiving periodontal treatment. The usual clinical periodontal parameters were recorded. The biofilm was analyzed by polymerase chain reaction quantitative. The risk factors of severe erosive gingivitis and severe periodontitis were assessed using Chi-square or Fischer's exact test were used.ResultsWhatever the medical and periodontal conditions of the patients, the results showed the existence of three main communities of periodontopathic, dysbiotic bacteria. The first including Tannnerella forsythia, Peptostreptococcus micros, Fusobacterium nucleatum, and Campylobacter rectus, was found in 100% of the patients, the second enriched with Treponema denticola in 60% and the third enriched with Porphyromonas gingivalis and Prevotella intermedia in 26%. Furthermore, there was a significant positive link between the duration of gMMP and the severity of erosive gingivitis (p = 0.009), and the loss of deep periodontal tissue (p = 0.04).ConclusionThis pilot study suggests a high periodontal risk in gMMP patients. The pathological processes, autoimmune on the one hand and plaque-induced on the other, may amplify each other. The application of periodontal therapy is therefore necessary in parallel with medical treatment. Nevertheless, further controlled studies are required to validate and complement these preliminary results.
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