Pemphigus vegetans – immunopathological findings in a rare variant of pemphigus vulgaris

A patient with painful erosions of the oral cavity and the labia minora developed multifocal blisters in inter‐triginous areas. These blisters eroded and evolved into papillomatous erosive vegetations. Histopathology and immunopathological investigations confirmed the diagnosis of pemphigus vegetans, mediated by IgG autoantibodies. The circulating IgG1 and IgG4 autoantibodies were exclusively directed against desmoglein 3, as shown by ELISA and indirect immunofluorescence studies. These IgG1 and IgG4 isotypes were also in vivo bound, as demonstrated with immunoperoxidase staining of perilesional skin. Our clinical, biochemical and immunopathological observations confirm the hypothesis that pemphigus vegetans is a variant of pemphigus vulgaris.     

The epidemiology of lip cancer: a review of global incidence and aetiology

Lip cancer (140 ICD-9) is a form of oral cancer that has a distinctive global epidemiology. This review summarises global incidence rates for male and female lip cancer with the aid of cancer atlases. High male lip cancer rates are reported for regions of North America (12.7 per 100 000 per annum), Europe (12.0 per 100 000 per annum) and Oceania (13.5 per 100 000 per annum), while it is virtually unknown in parts of Asia. Factors commonly cited as important in the aetiology of lip cancer include solar radiation, tobacco smoking and viruses. An attempt is made to summarise the evidence for factors that may be important in lip carcinogenesis. While incidence rates are generally stable or falling among males worldwide, they are rising in many female populations. The aetiology of the disease is far from established and much information regarding its pathogenesis is based on anecdotal rather than case-controlled epidemiological evidence. The epidemiology of lip cancer supports the proposal that the lip should be considered as a distinct cancer site, rather than being included with other forms of intraoral cancer.     

Soluble tumour necrosis factor (TNF)-receptor levels in serum as markers of anti-viral host reactivity.

The role of soluble receptors for TNF-alpha (sTNF-Rs) as markers of virus-induced host responses was studied by the use of murine model infections. A marked elevation in serum levels of sTNF-R75, but not sTNF-R55, was found 1 day after infection with vesicular stomatitis virus (VSV). In mice infected with lymphocytic choriomeningitis virus (LCMV), an early increase was also revealed, but peak levels of sTNF-R75 were observed later temporally related to maximal T cell-mediated anti-viral activity. Analysing different well characterized knockout mice, it was found that elevated release of sTNF-R75 into serum early after VSV infection was independent of T cells, whereas interferon (IFN)-alpha/beta seemed to be a major mediator. In contrast, increased release of sTNF-R75 into serum 8 days post-LCMV infection was mediated via T cells but independently of both CD40 ligand and IFN-gamma. A simple correlation between release of sTNF-Rs in vivo and macrophage activation in vitro was not present. These findings indicate that sTNF-R75 is indeed a sensitive marker of both innate and specific cell-mediated host reactivity during viral infection, but it is not correlated to a single immunological parameter.     

Longitudinal Change of Clinical and Biological Measures in Early Parkinson's Disease: Parkinson's Progression Markers Initiative Cohort

Objective: The objective of this study was to assess longitudinal change in clinical and dopamine transporter imaging outcomes in early, untreated PD. Methods: We describe 5‐year longitudinal change of the MDS‐UPDRS and other clinical measures using results from the Parkinson's Progression Markers Initiative, a longitudinal cohort study of early Parkinson's disease (PD) participants untreated at baseline. We also provide data on the longitudinal change in dopamine transporter 123‐I Ioflupane striatal binding and correlation between the 2 measures. Results: A total of 423 PD participants were recruited, and 358 remain in the study at year 5. Baseline MDS‐UPDRS total score was 32.4 (standard deviation 13.1), and the average annual change (assessed medications OFF for the treated participants) was 7.45 (11.6), 3.11 (11.7), 4(11.9), 4.7 (11.1), and 1.74(11.9) for years 1, 2, 3, 4, and 5, respectively (P < .0001 for the change over time), with a steeper change in year 1. Dopaminergic therapy had a significant effect on the change of MDS‐UPDRS. There was a significant longitudinal change in dopamine transporter binding in all striatal regions (P < .001). There was a significant but weak correlation between MDS‐UPDRS and dopamine transporter binding at baseline and years 1, 2, and 4, but no correlation between the rate of change of the 2 variables. Conclusions: We present 5‐year longitudinal data on the change of the MDS‐UPDRS and other clinical and dopamine transporter imaging outcome measures in early PD. These data can be used for sample size estimates for interventional studies in the de novo PD population. © 2018 The Authors. Movement Disorders published by Wiley Periodicals, Inc. on behalf of International Parkinson and Movement Disorder Society.     

Individualized home-monitoring of disease activity in adult patients with inflammatory bowel disease can be recommended in clinical practice: A randomized-clinical trial

BACKGROUND The optimal way to home-monitor patients with inflammatory bowel disease(IBD) for disease progression or relapse remains to be found.AIM To determine whether an electronic health(e Health) screening procedure for disease activity in IBD should be implemented in clinical practice, scheduled every third month(3 M) or according to patient own decision, on demand(OD).METHODS Adult IBD patients were consecutively randomized to 1-year open-label e Health interventions(3 M vs OD). Both intervention arms were screening for disease activity, quality of life and fatigue and were measuring medical compliance with the constant care web-application according to the screening interventions OD or3 M. Disease activity was assessed using home measured fecal calprotectin(FC)and a disease activity score.RESULTS In total, 102 patients were randomized(n = 52/50 3 M/OD) at baseline, and 88 patients completed the 1-year study(n = 43 3 M; n = 45 OD). No difference in the two screening procedures could be found regarding medical compliance(P =0.58), fatigue(P = 0.86), quality of life(P = 0.17), mean time spent in remission(P 0.32), overall FC relapse rates(P = 0.49), FC disease courses(P = 0.61), FC time to a severe relapse(P = 0.69) and remission(P = 0.88) during 1 year. Median(interquartile range) numbers of FC home-monitoring test-kits used per patient were significantly different, 3 M: 6.0(5.0-8.0) and OD: 4.0(2.0-9.0), P = 0.04.CONCLUSION The two e Health screening procedures are equally good in capturing a relapse and bringing about remission. However, the OD group used fewer FC home testkits per patient. Individualized screening procedures can be recommended for adult IBD patients in clinical web-practice.