Extended Inflammation Parameters (EIP) as Markers of Inflammation in Systemic Sclerosis.

Background: Systemic sclerosis (SSc) is an autoimmune disease characterized by inflammation, progressive vasculopathy, and fibrosis of skin and internal organs. The aim of the study was to evaluate extended inflammatory parameters (EIP) in patients with SSc in comparison to the control group of healthy subjects.

Methods: A total of 28 patients with SSc and 29 healthy controls (HCs) were included in the study.

Serum Levels of Selected IL-1 Family Cytokines in Patients with Morphea.

Morphea/localized scleroderma (LoS) represents an inflammatory-sclerotic skin disease, the pathogenesis of which is not fully understood. Given the important role of IL-1 family cytokines in the development and therapy of inflammatory diseases, including systemic sclerosis, we analyzed the clinical significance of serum levels of selected IL-1 family cytokines (IL-1α, IL-1β, IL-18, IL-33, IL-37 and IL-38) in LoS patients (n = 30) using the standardized disease assessment tools and comparison to healthy controls (n = 28). We also compared the pre- and post-treatment concentrations, i.

Standardized nailfold capillaroscopy in children with rheumatic diseases: a worldwide study.

Objectives: To standardly assess and describe nailfold videocapillaroscopy (NVC) assessment in children and adolescents with juvenile rheumatic and musculoskeletal diseases (jRMD) vs healthy controls (HCs).

Material And Methods: In consecutive jRMD children and matched HCs from 13 centres worldwide, 16 NVC images per patient were acquired locally and read centrally per international consensus standard evaluation of the EULAR Study Group on Microcirculation in Rheumatic Diseases. A total of 95 patients with JIA, 22 with JDM, 20 with childhood-onset SLE (cSLE), 13 with juvenile SSc (jSSc), 21 with localized scleroderma (lSc), 18 with MCTD and 20 with primary RP (PRP) were included.

Localized Blistering Eruption of the Face and Neck - A Case Study and Differential Considerations.

We describe a 36-year-old woman with erythematous lesions and well-tense blisters confined to the face and neck of two months history, without mucosal involvement and no triggering factors. A lesional skin biopsy showed a subepidermal blister. Direct immunofluorescence of peribullous skin identified linear deposits of IgG, IgA, and C3 complement along the basement membrane zone, whereas indirect immunofluorescence was negative.

Preliminary observations on ANGPT1 and ANGPT2 polymorphisms in systemic sclerosis: ANGPT2 rs2442598 and rs3739390 are associated with disease susceptibility and diffuse disease subtype.

Introduction: Angiopoietin-1 (Ang-1) and -2 (Ang-2) concentrations were found to be associated with systemic sclerosis (SSc).

Objectives: We explored whether single nucleotide polymorphisms of Ang-1 (ANGPT1) and Ang-2 (ANGPT2) genes can predict SSc susceptibility in Polish Caucasian patients.

Patients And Methods: Genotyping by reverse transcriptase–polymerase chain reaction and Sanger sequencing was performed in 48 patients with SSc and 38 controls.

Laser Therapy for the Treatment of Morphea: A Systematic Review of Literature.

Morphea, also known as localized scleroderma (LoS), comprises a set of autoimmune sclerotic skin diseases. It is characterized by inflammation and limited thickening and induration of the skin; however, in some cases, deeper tissues might also be involved. Although morphea is not considered a life-threatening disease, the apparent cosmetic disfigurement, functional or psychosocial impairment affects multiple fields of patients' quality of life.

Anti-endothelial cell antibodies are associated with apoptotic endothelial microparticles, endothelial sloughing and decrease in angiogenic progenitors in systemic sclerosis.

Introduction: Evidence has accumulated for the role of endothelial damage in systemic sclerosis (SSc) and the anti-endothelial cell antibodies (AECAs) might underlie vascular injury.

Aim: Since endothelial microparticles (EMPs) and circulating endothelial cells (CECs) reflect endothelial damage, we aimed to investigate their possible relationship with AECAs in SSc. We examined whether AECAs could affect endothelial repair based on the number of endothelial progenitor cells (EPCs).

T6746C and P72R Polymorphisms Are Associated with the Susceptibility to Diffuse Cutaneous Systemic Sclerosis.

Unlabelled: . NOTCH pathway and TP53 protein are involved in the development of fibrosis and autoimmune disorders, respectively. The aim of this study was to evaluate the role of single nucleotide polymorphisms (SNPs) of and genes and serum anti-TP53 antibodies with the susceptibility, clinical subset of systemic sclerosis (SSc), and clinical profile of SSc patient, particularly with lung involvement and disease activity.

Visfatin and chemerin levels correspond with inflammation and might reflect the bridge between metabolism, inflammation and fibrosis in patients with systemic sclerosis.

Introduction: Adipokines are regulatory molecules which act as mediators of the inflammatory, fibrotic and metabolic processes by interacting with the immune system.

Aim: We hypothesized that chemerin and visfatin by pro-inflammatory properties play a significant role in inflammation in systemic sclerosis. To address this hypothesis, we determined serum chemerin and visfatin levels in SSc patients, compared with the control group and defined the correlations with clinical and laboratory parameters in SSc patients.

A novel miRNA-4484 is up-regulated on microarray and associated with increased MMP-21 expression in serum of systemic sclerosis patients.

Systemic sclerosis (SSc) is a complex, heterogeneous connective tissue disease, characterized by fibrosis and ECM deposition in skin and internal organs, autoimmunity, and changes in the microvasculature. Profiling of circulating miRNAs in serum has been found to be changed in pathological states, creating new possibilities for molecular diagnostics as blood-based biomarkers. This study was designed to identify miRNAs that are differentially expressed in SSc and might be potentially contributing to the disease etiopathogenesis or be used for diagnostic purposes.

Application of superluminescent diodes (sLED) in the treatment of scarring alopecia - A pilot study.

Introduction: Photobiomodulation therapy with the use of light-emitting diodes (LEDs) is a fast growing therapeutic technique with a wide range of dermatologic indications. Recently it has been suggested that LED therapy could be beneficial in scarring alopecia.

Aim: Assessment of the efficacy of novel superluminescent diodes (sLED) with a "soft- start" as an adjuvant treatment in selected types of scarring alopecia.

Clinical associations of serum leptin and leptin/adiponectin ratio in systemic sclerosis.

Introduction: Leptin and adiponectin have recently received the attention of researchers as attractive biomarkers in systemic sclerosis (SSc) because of their role in the inflammatory process, vascular function and fibrosis. We hypothesized that leptin and adiponectin may be associated with disease activity and severity in patients with SSc.

Aim: To compare serum leptin, adiponectin and leptin/adiponectin levels in patients with SSc and healthy controls and to evaluate their possible relationship with frequently used laboratory markers and clinical findings.

Acyltransferase skinny hedgehog regulates TGFβ-dependent fibroblast activation in SSc.

Objectives: Systemic sclerosis (SSc) is characterised by aberrant hedgehog signalling in fibrotic tissues. The hedgehog acyltransferase (HHAT) skinny hedgehog catalyses the attachment of palmitate onto sonic hedgehog (SHH). Palmitoylation of SHH is required for multimerisation of SHH proteins, which is thought to promote long-range, endocrine hedgehog signalling.

Imbalanced serum levels of Ang1, Ang2 and VEGF in systemic sclerosis: Integrated effects on microvascular reactivity.

Introduction And Aim: Microangiopathy is a hallmark of systemic sclerosis (SSc). It is a progressive process from an early inflammatory and proangiogenic environment to insufficient microvascular repair with loss of microvessels. The exact underlying mechanisms remain ill-defined.

Anti-endothelial cell antibodies do not correlate with disease activity in systemic sclerosis.

Introduction: Anti-endothelial cell antibodies (AECA) recognize endothelial cell proteins and are thought to play an important role in vascular damage observed in systemic scleroderma (SSc) and many other autoimmune diseases. In SSc, AECA were found to be more common in patients with pulmonary hypertension, digital ulcers and nailfold capillaroscopic changes. Until now, there have been no studies examining the association between AECA positivity with the activity and duration of the disease.

Photodynamic therapy with the use of superluminescent diodes (sLED) in the treatment of actinic keratosis.

Background: Superluminescent diodes (sLED) appear to be an innovative and promising light source in photodynamic therapy (PDT), especially in actinic keratosis (AK) lesions treatment.

Aim: Assessment of tolerability and efficacy of sLED in topical 5-aminolevulinic acid (ALA) PDT of AK lesions.

Methods: 27 patients received ALA PDT with the use of sLED with "soft starter".

Resistin: a possible biomarker of organ involvement in systemic sclerosis patients?

Objectives: Resistin has strong pro-inflammatory and profibrotic properties, which are key pathogenetic processes in systemic sclerosis. We hypothesised that resistin may be associated with organ involvement and inflammatory process in SSc patients. To address this hypothesis, the aim of this study was to define serum resistin levels in SSc patients and control group and determine the correlation between this adipokine and internal organ involvement in SSc patients.

A 12-year-old Girl with Severe Plaque Psoriasis and Down Syndrome Treated Successfully with Etanercept.

The association between Down syndrome and psoriasis is unclear. Immunological abnormalities that present in individuals with Down syndrome result in mild immune debilitation, thus the risk of infectious complications during immunosuppressive therapy might be higher in this group of patients. We present a case of 12-year-old girl with severe plaque psoriasis and Down syndrome, who was initially treated with cyclosporine with good response.

Plasma endothelial microparticles reflect the extent of capillaroscopic alterations and correlate with the severity of skin involvement in systemic sclerosis.

Introduction And Aim: Endothelial microparticles (EMPs) are membrane-coated vesicles shed from endothelial cells and are considered markers of the endothelial state. It has been shown that total numbers of circulating EMPs are increased in patients with systemic sclerosis (SSc), but their clinical correlations have not yet been investigated in detail. We aimed to assess possible relationships between circulating EMPs and clinical as well as laboratory features among SSc patients with special attention to possible association with alteration in microvascular morphology objectified on nailfold videocapillaroscopy and clinical signs of microvascular complications.

The transcription factor GLI2 as a downstream mediator of transforming growth factor-β-induced fibroblast activation in SSc.

Objectives: Hedgehog signalling plays a critical role during the pathogenesis of fibrosis in systemic sclerosis (SSc). Besides canonical hedgehog signalling with smoothened (SMO)-dependent activation of GLI transcription factors, GLI can be activated independently of classical hedgehog ligands and receptors (so-called non-canonical pathways). Here, we aimed to evaluate the role of non-canonical hedgehog signalling in SSc and to test the efficacy of direct GLI inhibitors that target simultaneously canonical and non-canonical hedgehog pathways.

Evaluation of serum concentrations of the selected cytokines in patients with localized scleroderma.

Introduction: Localized scleroderma is an autoimmune disease primarily affecting the skin. The cause of disease remains unexplained although environmental factors are implicated, which are likely to be responsible for activation of the endothelium and subsequent inflammation leading to excessive synthesis of collagen and extracellular matrix components.

Aim: To determine concentrations of interleukin (IL)-27, transforming growth factor (TGF)-β1, TGF-β2, IL-6, and sIL-6R in patients with localized scleroderma compared to controls and to assess the relations between their levels and laboratory markers.

Immune system disturbances in Clouston syndrome.

Background: Clouston syndrome belongs to the family of ectodermal dysplasias. So far, a defective immune response has not been reported in Clouston syndrome. We report, for the first time, immunological particularities of a large multigenerational Polish family with Clouston syndrome.

High frequency ultrasonography of the skin and its role as an auxillary tool in diagnosis of benign and malignant cutaneous tumors--a comparison of two clinical cases.

The number of dermatologic entities that can be studied by ultrasound examination (US) of the skin is increasing. Conventional US and high frequency US (HFUS) are considered useful additional tools in improving the diagnosis and management of common benign and malignant skin tumors. US may help in positive and differential diagnosis of primary melanocytic neoplasms and of locoregional spread in melanoma patients.