Amyopathic dermatomyositis may be on the spectrum of autoinflammatory disease: A clinical review.

Systemic autoinflammatory diseases (SAIDs) are distinct from autoimmune diseases. The former primarily results from abnormal innate immune response and genetic testing is crucial for disease diagnosis. Similar cutaneous involvement is a main feature for both SAID and dermatomyositis (DM), so they can be confused with each other. A literature search of PubMed and MEDLINE was conducted for relevant articles. The similarities and differences between these two types of diseases were analyzed. We found phenotypic similarities between these two types of disorders. Accumulating data supports a major role of the innate immune system and a similar cytokine profile. Molecular testing using an autoinflammatory disease gene panel may help identify SAID patients from the DM population and may offer therapeutic benefit using interleukin-1 (IL-1) inhibitors. A subset of DM, notably amyopathic dermatomyositis in the absence of autoantibodies may be on the spectrum of autoinflammatory disease.

Prevalence of diabetes mellitus in bullous pemphigoid patients in the absence of dipeptidyl peptidase-4 inhibitors: a systematic review and meta-analysis.

Bullous pemphigoid (BP) has been associated with dipeptidyl peptidase-4 inhibitor (DDP-4i) use in patients with diabetes mellitus (DM). The prevalence and association of DM in BP patients independent of DPP-4i use has not been investigated by meta-analysis. To perform a systematic review and meta-analysis on the association between diabetes and bullous pemphigoid. The goal was to determine the prevalence and pooled odds ratio of BP patients with DM in the absence of DDP-4i use compared to the general population prevalence of diabetes mellitus. OVID Medline, EMBASE, Cochrane Central and Web of Science were searched for relevant studies published from inception to April 2020. Case-control, case-series, cohort, and cross-sectional studies that included the association of BP and DM without DDP-4i's, in any language. PRISMA guidelines were followed for data extraction and the Newcastle-Ottawa Scale for risk of bias evaluation. Three reviewers independently performed data extraction. Pooled odds ratio and prevalence were calculated using the random effects model. The odds ratio and prevalence of BP patients with DM. Overall, 8 studies out of 856 identified publications through data base searches were included. The pooled prevalence of diabetes in patients with BP was 20.0% [95% CI 14%-26%; p = 0.00]. Within the comparative non-BP control population, 13% had diabetes. BP patients were more likely to have diabetes compared to a control population without BP [OR 2.10, 95% CI 1.22-3.60; p = 0.01]. This study found that twice the number of BP patients have DM (20%) compared to the general population reported as 10.5%, warranting monitoring of blood glucose levels in BP patients who may have yet undeclared or undiagnosed DM when initiating systemic steroids.

Vesicular Eruption Secondary to Bites by Larval Amblyomma americanum.

Tick-borne illness is an increasingly concerning cause of human infectious disease. Not only do ticks transmit disease, but their bites also may cause impressive local reactions. This report highlights a case of a widespread vesicular eruption secondary to bites by larval Amblyomma americanum sustained by a 58-year-old woman. This case posed a diagnostic challenge because of the unusually large number and wide distribution of bites as well as the subsequent vesicular reaction that ensued. It is necessary for dermatologists in tick-endemic areas to keep tick bites in the differential when evaluating vesicular eruptions. In addition, it is important for dermatologists to be aware of the tick species in their area.

SARS-CoV-2/COVID-19 and its relationship with NOD2 and ubiquitination.

COVID-19 infection activates the immune system to cause autoimmune and autoinflammatory diseases. We provide a comprehensive review of the relationship between SARS-CoV-2, NOD2 and ubiquitination. COVID-19 infection partly results from host inborn errors and genetic factors and can lead to autoinflammatory disease. The interaction between defective NOD2 and viral infection may trigger NOD2-associated disease. SARS-CoV-2 can alter UBA1 and abnormal ubiquitination leading to VEXAS syndrome. Both NOD2 and ubiquitination play important roles in controlling inflammatory process. Receptor interacting protein kinase 2 is a key component of the NOD2 activation pathway and becomes ubiquitinated to recruit downstream effector proteins. NOD2 mutations result in loss of ubiquitin binding and increase ligand-stimulated NOD2 signaling. During viral infection, mutations of either NOD2 or UBA1 genes or in combination can facilitate autoinflammatory disease. COVID-19 infection can cause autoinflammatory disease. There are reciprocal interactions between SARS-CoV-2, NOD2 and ubiquitination.

Alternative uses of ustekinumab for non-indicated dermatological conditions: a systematic review.

Ustekinumab is approved for the treatment of psoriasis and Crohn's disease. Because many dermatological conditions are due to immune-mediated development, ustekinumab may be effective in other conditions. A systematic review of the off-label uses of ustekinumab, as well as on-label adverse effect, was performed, reporting on clinical improvement. MEDLINE, Embase, Web of Science, and Cochrane databases were searched for studies regarding ustekinumab treatment of rativa (HS), lichen planus (LP), pyoderma gangrenosum (PG), pityriasis rubra pilaris (PRP), cutalopecia areata (AA), atopic dermatitis (AD), Bechet's disease, bullous pemphigoid (BP), hidradenitis suppuaneous sarcoidosis, cutaneous systemic lupus erythematosus (SLE), and vitiligo. Descriptive statistics were performed. 74 articles of 4596 screened were included, and reported on 212 patients receiving ustekinumab treatment. Across all studies, ustekinumab showed promise in treating patients: AA (10/12 patients; 83.3% improvement), AD (28/74 patients; 37.8% improvement), HS (42/52 patients; 80.8% improvement), and PRP (25/27 patients; 92.6% improvement), among others. Adverse events were noted with the use of ustekinumab, including development of AA (four patients), AD (three patients), and BP (four patients), among others. Ustekinumab can be a promising option for patients with dermatological conditions refractory to traditional therapies. Adverse events must be monitored in certain patients.