Diffuse fasciitis with eosinophilia in boys: a case-based review.

Diffuse fasciitis with eosinophilia (EF) is a rare condition classified as a part of the connective tissue disorders. The clinical presentation of this condition can be diverse, however the main symptoms include symmetrical swelling and hardening of distal parts of limbs accompanied by peripheral eosinophilia. The diagnostic criteria are not specified.

The impact of single nucleotide polymorphisms in and genes on disease activity and the presence of methotrexate treatment side effects in children with juvenile idiopathic arthritis.

Introduction: To determine the impact of single nucleotide polymorphisms (SNPs) in MTHFR and MTRR genes on disease activity and the presence of MTX therapy adverse events in Polish children with juvenile idiopathic arthritis (JIA).

Methods: SNP genotyping was performed using genomic DNA isolated from peripheral blood samples.

Results: Patients with MTHFR rs1801133 CT/TT variant had higher values of inflammatory markers, number of joints with active arthritis, and JADAS-71 value at the baseline of MTX treatment.

variants as predictors of methotrexate-related toxicity in children with juvenile idiopathic arthritis.

: Methotrexate (MTX) administered at the dose 10-15 mg/m is currently recommended as the first line therapy in most juvenile idiopathic arthritis (JIA) subtypes. Gastrointestinal side effects and hepatotoxicity are the most prevalent manifestations of MTX intolerance, frequently leading to discontinuation of otherwise effective treatment. Genetic variability within solute carrier organic anion transporter family member 1B1 (), encoding a hepatic MTX membrane transporter, has been associated with high-dose MTX efficacy and toxicity in paediatric patients with acute lymphoblastic leukaemia.

The impact of single nucleotide polymorphisms in ADORA2A and ADORA3 genes on the early response to methotrexate and presence of therapy side effects in children with juvenile idiopathic arthritis: Results of a preliminary study.

Aim: Methotrexate (MTX) administered at the dose 10-15 mg/m is recommended as the first-line therapy in most juvenile idiopathic arthritis (JIA) subtypes. The disease-modifying effect of methotrexate is associated with release of adenosine and mediated via binding to adenosine receptor A2A (ADORA2A) and 3 (ADORA3). The aim of our study was to determine the association between single nucleotide polymorphisms in ADORA2A (rs2236624, rs2298383) and ADORA3 (rs3393) receptor genes on the disease activity and presence of MTX therapy side effects in patients with JIA.

Prefilled pen versus prefilled syringe: a pilot study evaluating two different methods of methotrexate subcutaneous injection in patients with JIA.

Background: Methotrexate is the most commonly used disease-modifying antirheumatic drug recommended in the treatment of juvenile idiopathic arthritis. It can be administered orally or subcutaneously, the latter method is associated with fewer side effects and higher drug bioavailability. Nevertheless, the pain associated with injection is a considerable drawback of this treatment option in the pediatric population.

Tocilizumab in the treatment of systemic-onset juvenile idiopathic arthritis - single-centre experience.

Objectives: The aim of the study was to evaluate the efficacy and long-term safety of tocilizumab treatment in children with systemic-onset juvenile idiopathic arthritis in a single centre.

Material And Methods: The study was based on a retrospective analysis of a cohort of 10 patients with systemic-onset juvenile idiopathic arthritis who were treated with tocilizumab in the period September 2011-July 2017. Their medical records were analysed taking into consideration the effectiveness of tocilizumab treatment and frequency of side effects.

From fibrosis to diagnosis: a paediatric case of microscopic polyangiitis and review of the literature.

We herein describe a case of a 6-year-old girl diagnosed with microscopic polyangiitis (MPA) after a long diagnostic process demanding the cooperation of paediatric respiratory medicine specialists, rheumatologists, nephrologists and radiologist. First symptoms of MPA were observed after Mycoplasma pneumoniae infection and included persistent crepitations at the basis of both lungs and mild haematuria. CT imaging showed features of lung fibrosis, renal biopsy was indicative of mesangial proliferative glomerulonephritis, in the skin biopsy features of microscopic polyangiitis were described.

In the Pursuit of Methotrexate Treatment Response Biomarker in Juvenile Idiopathic Arthritis-Are We Getting Closer to Personalised Medicine?

Purpose Of Review: Methotrexate (MTX) is the most widely used disease-modifying antirheumatic drug (DMARD) in paediatric rheumatology and the mainstay in the therapy of juvenile idiopathic arthritis (JIA). Despite its common use, about 30% of children fail to respond to this medicine that results in potentially irreversible joint damage.

Recent Findings: No clinical biomarker that would predict the outcome of MTX therapy exists.

The role of polymorphism of , -, and α genes in cutaneous T-cell lymphoma pathogenesis.

Introduction: As the pathogenesis of cutaneous T-cell lymphomas (CTCL) is not fully understood, inherited gene polymorphisms are considered to play a role in the development of lymphomas.

Aim: To investigate whether certain gene polymorphisms might be involved in the development of CTCL.

Material And Methods: In the case-control study we compared the frequency of nine selected single nucleotide polymorphisms (SNP) of seven genes (α and and α) in 43 CTCL and Polish cases using the amplification refractory mutation system polymerase chain reaction method.

Kaleidoscope of autoimmune diseases in HIV infection.

Within the last 30 years, the human immunodeficiency virus (HIV) infection has changed its status from inevitably fatal to chronic disorder with limited impact on life span. However, this breakthrough was mainly the effect of introduction of the aggressive antiviral treatment, which has led to the clinically significant increase in CD4+ cell count, resulting in fewer cases of the acquired immunodeficiency syndrome (AIDS) and improved management of opportunistic infections occurring in the course of the disease. The occurrence of a particular autoimmune disease depends on degree of immunosuppression of the HIV-positive patient.

The paediatric rheumatologist and orphan disease - a story without happy ending.

Orphan diseases are not a common challenge in the everyday practice of the rheumatologist. Despite their extremely rare occurrence one of the patients under our care developed one of them - neuronal ceroid lipofuscinosis, the most frequent neurodegenerative disease observed in the paediatric population. We report a case of 2-year-old girl diagnosed with oligoarticular form of juvenile idiopathic arthritis treated in our Department with steroids and methotrexate and staying in the stage of disease remission.

Frequency of streptococcal upper respiratory tract infections and HLA-Cw*06 allele in 70 patients with guttate psoriasis from northern Poland.

Introduction: The association of guttate psoriasis with a streptococcal throat infection and HLA-Cw*06 allele is well established in different populations. Nevertheless, only few studies on this form of disease have been performed in the Polish population.

Aim: To analyze the frequencies of streptococcal-induced guttate psoriasis and HLA-Cw*06 allele in 70 patients with guttate psoriasis originating from northern Poland.

Superiority of ivermectin 1% cream over metronidazole 0·75% cream in treating inflammatory lesions of rosacea: a randomized, investigator-blinded trial.

Background: Few therapeutic alternatives currently exist in the treatment of papulopustular rosacea (PPR).

Objectives: To demonstrate superiority of once-daily ivermectin 1% cream (IVM 1%) once daily vs. twice-daily metronidazole (MTZ 0·75%) cream, regarding percentage reduction of inflammatory lesions in subjects with moderate to severe PPR.

Chemokines and cytokines network in the pathogenesis of the inflammatory skin diseases: atopic dermatitis, psoriasis and skin mastocytosis.

Chemokines are signaling peptides which regulate cell trafficking and provide control of the tissue-specific cell homing. In the skin, chemokines are secreted both by the resident cells such as keratinocytes, melanocytes, fibroblasts, dendritic cells and mast cells, as well as by infiltrated cells - lymphocytes, eosinophils, and monocytes. Chemokines, together with cytokines, participate in induction and maintenance of inflammation in the skin and regulate the composition of the cellular infiltrates.

Safety profile of antimicrobial peptides: camel, citropin, protegrin, temporin a and lipopeptide on HaCaT keratinocytes.

Antimicrobial peptides (AMPs) are an essential part of the innate immunity of the skin and mucosal surfaces. They have a broad spectrum of antimicrobial activity: antibacterial, antifungal, antiviral as well as antiprotozoal. Numerous studies using AMPs as potential agents against different microbes has been performed during the last two decades.

Interleukin-13 promoter gene polymorphism -1112 C/T is associated with atopic dermatitis in Polish patients.

Interleukin 13 (IL-13) is one of the major cytokines involved in the IgE synthesis in patients with atopic dermatitis (AD). IL-13 gene has been mapped on chromosome 5q31-33 region associated with atopic conditions, where several polymorphisms are described. The aims of the study were to establish the frequency of the IL-13 gene polymorphism and its relation to serum IgE and IL-13 levels and SCORAD.

Childhood nail alterations in Polish population.

The epidemiology and nature of childhood nail apparatus pathology is not well known. The aim of our study was to investigate the frequency and nature of nail alterations in Polish pediatric patients. Among 1588 patients diagnosed and treated at our clinic due to nail alterations, 82 (5.

Activity of Antimicrobial Peptides and Conventional Antibiotics against Superantigen Positive Staphylococcus aureus Isolated from the Patients with Neoplastic and Inflammatory Erythrodermia.

Superantigens are proteins comprising a group of molecules produced by various microorganisms. They are involved in pathogenesis of several human diseases. The aim of the study was the comparison of susceptibility to antibiotics and antimicrobial peptides (AMPs) of Staphylococcus aureus (SA) strains producing staphylococcal enterotoxins SEA, SEB, SEC, SED, and TSST-1 and nonproducing ones.

A retrospective study of 12 cases of pyoderma gangrenosum: why we should avoid surgical intervention and what therapy to apply.

Lesions of skin are ubiquitous in the medical field. The varying etiopathologies with similar presentation can pose a misleading picture, especially when faced with less common skin diseases. Furthermore, the misdiagnosis can cause detrimental effects on the patient's morbidity and mortality, which was seen in the case series study we performed on pyoderma gangrenosum.

Association of psoriasis with the VEGF gene polymorphism in the northern Polish population.

Background: Neovascularization plays an important role in pathogenesis of psoriasis and vascular endothelial growth factor (VEGF) seems to be the main angiogenic factor involved in this disease. Published studies which analysed the role of VEGF gene polymorphism in psoriasis were limited and they received controversial results. Objective The aim of our study was to evaluate the association between -1154 G/A, -460 T/C and +405 G/C polymorphisms and the psoriasis susceptibility and to determine whether this genetic variation influence levels of VEGF protein expression.

Relationship between serum levels of interleukin-18, IgE and disease severity in patients with atopic dermatitis.

Background: Interleukin (IL)-18 is a pleiotropic cytokine. Synergistically with IL-12, IL-18 promotes immune responses of the T helper type, by enhancing synthesis of interferon-γ and inhibiting IgE production. IL-18 can also enhance production of IL-4 and IL-13 production, and stimulate synthesis of IgE.

Dermatitis artefacta--a long way from the first clinical symptoms to diagnosis.

Dermatitis artefacta is a disease that occurs as a result of a self-inflicted injury to the skin. The problem quite often is undiagnosed for a long time until the clinical look of bizarre skin lesions combined with non-specific histology and normal blood tests lead to the final identification. This report presents the case of a 62-year-old man who was diagnosed after 10 years of duration of disease.