Bullous pemphigoid (BP) is an autoimmune subepidermal blistering disease affecting mainly the elderly. The subtype of the disease induced by physical agents represents a rare and, therefore, insufficiently characterized form. In the present study, we aimed to contribute to a better understanding of the pathogenetic mechanisms involved in the onset of BP induced by different trigger factors. We have retrospectively analyzed nine cases of BP. All patients were characterized based on clinical, epidemiological and immunological parameters. For each case, the trigger factor involved was specified. In addition to our retrospective analysis, a comprehensive review of the 59 published cases was conducted, regarding the involvement of trigger factor in BP, and clinical, epidemiological and immunological data were collected. In the local study, conducted on nine patients diagnosed with BP, various trigger factors were identified: contrast substance injection, surgical procedure, mechanical trauma, insect bite, thermal burn, radiotherapy and ultraviolet exposure associated with pre-existing psoriasis. The autoantibodies from all patients were shown to activate granulocytes and induce dermal-epidermal split. Different hypotheses regarding the pathogenetic mechanism involving the trigger factors have been discussed. In regard of the pathogenetic mechanism, we believe that the most reliable hypothesis is that BP patients already have low titers of anti-basement membrane autoantibodies which activate the granulocytes. However, more studies are needed for a better understanding of the pathogenetic mechanism of the intervention of trigger factors.

BackgroundFactors associated with survival in pemphigus have not yet been thoroughly addressed. Therefore, in the present study, risk factors for overall mortality in a large group of patients with pemphigus vulgaris and foliaceus were investigated.MethodsA retrospective hospital-based cohort study was carried out, between October 1998 and November 2012, in the Department of Dermatology of the University of Medicine and Pharmacy “Iuliu Hatieganu”, Cluj-Napoca, Romania. The investigated prognostic endpoint was the overall survival of the patients.ResultsA total of 130 patients were studied (108 with pemphigus vulgaris and 22 with pemphigus foliaceus). In pemphigus vulgaris group, univariate analysis found a statistically significant association between the age of onset ≥ 65 years (p < 0.001), presence of coronary heart disease (p = 0.006), presence of cardiac arrhythmia (p = 0.004), level of anti-desmoglein1 autoantibodies ≥ 100 U/mL (p = 0.047) at diagnosis and the survival of the patients. An age-adjusted analysis showed significant results for coronary heart disease. Multivariate analysis identified the age of onset ≥ 65 years and the presence of coronary heart disease at diagnosis as independent risk factors associated with overall mortality. In patients with pemphigus foliaceus, age of onset ≥ 65 years (p = 0.021) was associated with poor survival.ConclusionsIn addition to common prognostic factors, including older age and cardiovascular comorbidities, level of autoantibodies was found to be a disease-specific factor associated with overall mortality in pemphigus vulgaris. The newly identified factors have major implications for the stratification of patients and should greatly facilitate further epidemiological studies in pemphigus. In addition, they provide useful information for the design of personalized therapeutic plans in the clinical setting.

We have estimated, the total number of EB patients in Romania and we have estimated the incidence and the prevalence of EB. We have also managed to approximate the distribution of EB types in Romania. Moreover, we performed a phenotypic and genotypic characterization in some of the patients included in the EB register.

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.Electronic supplementary materialThe online version of this article (10.1186/s13023-018-0855-x) contains supplementary material, which is available to authorized users.

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Lichen planus pemphigoides (LPP) and bullous lichen planus (BLP) are rare dermatoses, which are characterised by blisters and lichenoid lesions. Their clinical presentation is heterogenous, displaying overlapping features or mimicking other dermatological diseases. Therefore, diagnosis can often be challenging, requiring a thorough dermatological examination along with distinctive histological and immunopathological characteristics. Lichenoid degeneration of the basal epidermis exposes various antigens of the dermal-epidermal junction in LPP, resulting in the breakdown of immune tolerance, hence, the production of autoantibodies against type XVII collagen. Conversely, no pathogenic autoantibodies are detected in BLP. However, some cases of mucosal lichen planus might display immunopathological features suggestive of autoimmune blistering diseases. Therefore, a better understanding of the pathophysiology of these two distinct dermatoses is imperative. The aim of this review was to provide a summary of the current knowledge on the clinical hallmarks, diagnosis and available therapeutic options in LPP and BLP.

Background: Oral cancer is highly aggressive due to difficult diagnosis, therapy resistance and increasing frequency; thus finding prevention therapies is very important. Aim: This study evaluates the use of gold and silver nanoparticles (NPs), phyto-synthesized with Cornus mas extract against oral dysplastic lesions. Methods: NPs were characterized by UV–Vis, Fourier-transform infrared spectroscopy, transmission electron microscopy, x-ray diffraction and laser Doppler microelectrophoresis. Biological testing employed two human oral cell lines: gingival fibroblasts and dysplastic keratinocytes and evaluated viability, cell death mechanisms and cellular uptake. Results: NPs induced selective toxic effects against dysplastic cells. p53/BAX/BCL2 activation and PI3K/AKT inhibition led to cell death through necrosis and apoptosis. NPs also induced antioxidant and anti-inflammatory effects. Conclusion: NPs of gold and silver showed promising beneficial effects in the therapy of oral dysplasia.