• 2018-07
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  • br Contributors br Acknowledgements br Introduction Pemphigu


    Introduction Pemphigus disorders are characterized by autoantibodies binding to desmosomes between epidermal cells, resulting in the loss of cell to cell adhesion and blister formation. In pemphigus foliaceus, autoantibodies predominantly bind to desmoglein 1, while in PV, autoantibodies bind to desmoglein 3, with some patients also forming autoantibodies to desmoglein 1 [[1], [2], [3], [4]]. Antibodies targeting other keratinocyte proteins are also detected [5]. Exposure to desmoglein autoantibodies initiates cell signaling pathways resulting in keratinocytes losing their polygonal shape, detaching from neighboring cells and undergoing apoptolysis [6]. While histology shows few inflammatory cells, there is a significant amount of cell signaling activity caused by these autoantibodies, and numerous local and serum cytokine changes have been noted. When comparing desmoglein 3 reactive T-cells in human leukocyte antigen (HLA) matched family members compared to patients with pemphigus, a greater proportion of Th2 T-cells are seen, thus demonstrating the importance of differential downstream cytokine expression. The pathogenesis of BP begins with loss of tolerance and generation of molecular calculator targeting BP180 and BP230, transmembrane proteins in the hemidesmosomal complexes [7]. The inception of various pro-inflammatory mediators and chemoattractants recruit immune cells resulting in an inflammatory infiltrate of eosinophils, neutrophils, and lymphocytes [8]. Alterations in the BP180 protein can lead to keratinocyte thymic stromal lymphopoetin (TSLP) expression, which can result in substantial downstream signaling seen with an allergic phenotype [9].
    Conflict of interest
    Introduction Aortic dissection (AD) is a rare cardiovascular disease with high mortality, the incidence of which has increased, affecting progressively younger patients in recent years. The promotion of aortic replacement and interventional therapy in clinical practice has significantly reduced the mortality rate of patients with AD [1, 2]; however, both treatments have certain limitations. Aortic replacement is traumatic, costly, and can lead to a variety of serious clinical complications, bringing heavy psychological pressure and financial burden to patients and families. However, complications from interventional therapy are relatively rare and treatment is less expensive, but it is mostly suitable for Stanford B type cases and has a narrow range of adaptability. Therefore, an in-depth understanding of the pathogenesis of AD and the search for more effective and safe intervention targets are required for AD prevention and treatment and will have great clinical value and social significance. Vascular smooth muscle cells (SMCs) are an important component of aortic structure and play a vital role in maintaining normal structure and function of the aorta. In addition to maintaining vasoconstriction and diastolic function, SMCs can continuously synthesize and degrade the extracellular matrix (ECM) and maintain its dynamic balance [3]. In addition, vascular SMCs can also feel hemodynamic pressure and maintain cytoskeleton and ECM reconstruction [4]. Excessive loss of SMCs can lead to a variety of severe vascular diseases, including atherosclerosis, aortic aneurysm, and AD [[5], [6], [7]]. Significant reduction of SMCs was observed in human AD vascular tissues, and this change was associated with AD progression [8, 9]. Once AD occurred, the heavy chain of SMCs could be released into the blood and was found to be elevated in comparison with control subjects; this increase was of high value and specificity for the diagnosis of AD [10, 11]. In addition, evidence suggested that AD is pathologically related to degeneration of aortic media, characterized by loss, breakage and failure of SMCs [2, 12]. Therefore, excessive loss of SMCs in the aorta is an important cause of AD.