Demographic characteristics, aetiology, and assessment of treatment options in leukocytoclastic vasculitis

Introduction: Vasculitides are a heterogeneous group of disease characterized by inflammation of the blood vessel walls. Etiological factors include infections, drugs, connective tissue diseases, and malignancies.Aim: To examine the demographic characteristics, etiological factors, and treatment options in 75 patients with leukocytoclastic vasculitis.Material and methods: The study included 75 patients diagnosed with leukocytoclastic vasculitis at our clinic. The patients' medical records were reviewed to determine their age, sex, presence of systemic symptoms, possible etiological factors, laboratory results, types of cutaneous lesions, locations of the lesions, treatment options, and disease course.Results: There were 43 women and 32 men. Cutaneous lesions affected only the lower limbs in 60 of the 75 patients (80%) and usually presented as palpable purpura (64%, n = 48). Arthralgia (26.7%, n = 20) was the most frequent extracutaneous symptom. Of the patients with secondary vasculitis, the most common causes were infections and drugs. The mean age of the patients with Henoch-Schonlein purpura was 26.8 years. There was no significant association between age and renal, gastrointestinal, or joint involvement.Conclusions: The most common form of vasculitis in our study was cutaneous leukocytoclastic vasculitis. In most of the patients it appeared to be idiopathic. Among drugs, antibiotics were the most common etiological factor. In 4 patients, the cutaneous leukocytoclastic vasculitis behaved like the paraneoplastic syndrome

Mandibular actinomycosis mimicking tumor recurrence

Actinomycosis is a rare disease caused by a microorganism of the normal oral flora. Infection is characterized by swollen tissues and sinuses from which pus drains containing characteristic sulfur granules. Since actinomycosis is a tumorous infection of skin and subcutaneous tissue, this chronic lesion can occasionally mimic neoplasia. A male patient is presented; he had a tumor-like lesion of the left mental region that was initially diagnosed as tumor recurrence, but proved to be an Actinomyces infection on histopathologic examination. Excellent therapeutic response was obtained with a combination of antibiotic therapy, surgical debridement, and mandibular curettage. Vestibuloplasty-commissuroplasty was also performed

A rare dementing disease: Adult neuronal ceroid lipofuscinoses

To emphasize the importance of clinical and ultrastructural findings for diagnosing adult neuronal ceroid lipofuscinosis (ANCL), the authors retrospectively reviewed six patients with biopsyproven ANCL. In all cases, ophthalmologic examinations were normal, and electron microscopic studies demonstrated characteristic granular osmiophilic deposits within the eccrine epithelial cells. The inheritance and mechanism of ANCL remain unknown, and the diagnosis is based on clinical features and ultrastructural examination of the cerebral tissue or extracerebral accumulation of lipopigments. This study suggests that ANCL should be considered as a possible diagnosis in patients with early-onset dementia. © 2012 American Psychiatric Association

Association of NFKB1 and NFKBIA Polymorphisms in Relation to Susceptibility of Behcet's Disease

Behcet's disease (BD) is a chronic inflammatory autoimmune disease. Although raised levels of proinflammatory cytokines in BD have been reported, the pathogenesis is still unknown. The aim of this study was to investigate the association of NFKB1 and NFKBIA polymorphisms and their single and combined analysis effects on susceptibility of BD in Turkish population. We analysed the distribution of NFKB1 -94 ins/del ATTG (rs28362491) and NFKBIA 3 UTR AG (rs696) polymorphisms using PCR-RFLP method in 89 patients with BD and 190 controls in this population. Statistical analysis of the results was performed by calculating OR, and 95% CI via (2) test and using Bonferroni correction. According to the significant results of both single and combined genotype analysis, the frequencies of ins/ins genotype and ins allele of rs28362491 were significantly higher in patients with BD (Pc=0.003, 0.004, respectively). Also, higher frequencies of the rs696 variant containing AA genotype was found in patients with BD (Pc=0.0033), whereas no statistical significant differences in distribution of the alleles of rs696 polymorphism in patients and controls. In addition, according to the combined genotype analysis, the wild type of both rs28362491 and rs696 polymorphisms (ins/ins/AA genotype) was also significantly higher in BD cases (Pc=0.044). Our findings prove that both single and combined genotype analysis of rs28362491 and rs696 polymorphisms indicate that the wild genotypes of both two SNPs (ins/ins and AA genotypes) and ins/ins/AA combined genotype are strongly associated with enhanced risk of BD in a Turkish population

Association of Pre-miRNA-499 rs3746444 and Pre-miRNA-146a rs2910164 Polymorphisms and Susceptibility to Behcet's Disease

MiRNAs and NFKB1 are well-known immune response and inflammation regulators. MiRNA gene polymorphisms may affect miRNA biogenesis and function and, may thus, lead to changes in the expression of hundreds of genes such as NFKB1. The aim of this study was to investigate the association of Behcet's disease (BD) with NFKB1 rs28362491, pre-miRNA-146a rs2910164, and pre-miRNA-499 rs3746444 polymorphisms, as well as the analysis of their single and combined effects on its susceptibility in a Turkish population. These polymorphisms were analyzed by using the polymerase chain reaction-restriction fragment length polymorphism method in 100BD patients and 145 healthy control subjects. The results were analyzed statistically using Pearson chi-square ((2)) test and Fisher's exact test (two sided). According to genotype analysis, the frequencies of ins/ins genotype and ins allele of rs28362491 were considerably higher in BD patients. Also, miRNA-499 rs3746444 homozygous (TT) genotypes exibited a significantly higher risk in patients with BD (odds ratios [OR]=3.0, 95% confidence intervals [95% CI]=1.284-7.007, p=0.017). Moreover, the frequency of T allele of rs3746444 was a risk factor for BD (OR=1.562, 95% CI=1.087-2.24, p=0.015). In addition, significant differences were found between the groups concerning miRNA-146a rs2910164 polymorphism. Homozygous CC genotype and C allele of rs2910164 polymorphism were found to be protective factors against BD. The results of the combined genotype analysis showed no notable differences between the multiple comparisons of rs28362491-rs2910164 and of rs28362491-rs3746444 in patients and control groups. Our data demonstrate that homozygous CC genotype and C allele of rs2910164 polymorphism are protective factors against BD, but rs3746444 and rs28362491 polymorphisms in miRNA-499 and in NFKB1 promoter are involved in the genetic susceptibility of BD. In addition, TT and ins/ins genotypes may influence certain proinflammatory cytokines and, may thus, play a role in the pathogenesis of BD

Atopic dermatitis diagnosis and treatment consensus report

Atopic dermatitis (AD) is a chronic, itchy, and recurrent inflammatory skin disease. AD, which is known as a childhood disease because of its common occurrence, is also an important health problem in adults. With increasing prevalence rates throughout each year, particularly in developed countries, AD has a heterogeneous clinical presentation that varies with age and different degrees of severity. The treatment includes the use of topical or systemic agents after identifying the needs of the patients. Especially, the identification of molecules responsible for pathogenesis recently has allowed the development of tailored treatments. With a better understanding of both the disease and the economic burden of AD recently, studies have gained momentum on diagnosis, treatment, and quality of life. Guidelines and consensus reports addressing diagnostic and therapeutic approaches have been published in our country, too, in parallel to publications in various countries. In this age of rapid information sharing, all kinds of information need to be updated frequently and become further useful. For this purpose, it is planned to develop a current consensus guideline under the leadership of the Dermatoimmunology and Allergy Association, with the contributions of the Cosmetology and the Dermatology Academy Association, Kayseri Dermatology and the Venereal Diseases Association, and Manisa Dermatology and the Venereal Diseases Association, and through the participation of faculty members experienced in the diagnosis and treatment of AD. The topics and the authors were chosen in December 2020. All Medline data published in the years between 1980 and 2021, current AD diagnosis and treatment guidelines, meta-analytical studies, and expert opinions and experiences were reviewed, and section drafts were developed. Literature data and section drafts were assessed and discussed during a meeting held in March 2021 with the participation of all authors. Then, the sections were finalized via e-mail correspondences and submitted as a final consensus report

Omalizumab versus cyclosporin-A for the treatment of chronic spontaneous urticaria: Are there specific patient characteristics or biomarkers to indicate response to treatment?

...European Academy of Allergy and Clinical Immunolog

Omalizumab versus cyclosporin-A for the treatment of chronic spontaneous urticaria: Can we define better-responding endotypes?

Background: Chronic Spontaneous Urticaria (CSU) is characterized by recurrent wheals and/or angioedema for longer than 6-weeks. Guidelines recommend Omalizumab (Oma) as first-line and Cyclosporine-A (Cs-A) as second-line treatment in antihistamine resistant CSU. This step-wise algorithm might be time-consuming and costly. Objective: To determine indicators of response to Oma or Cs-A in CSU patients. Methods: We retrospectively analyzed data from seven centers in Turkey; the inclusion criteria for patients were to receive both Oma and Cs-A treatment (not concurrently) at some point in time during their follow-up. Clinical and laboratory features were compared between groups. Results: Among 110 CSU patients; 47 (42.7%) were Oma-responders, 15 (13.6%) were Cs-A-responders, and 24 (21.8%) were both Oma and Cs-A responders and 24 (21.8%) were non-responders to either drug. High CRP levels were more frequent in Cs-A-responders (72.7% vs. 40.3%; p = 0.055). Oma-responders had higher baseline UCT (Urticaria Control Test) scores (6 vs. 4.5; p = 0.045). Responders to both drugs had less angioedema and higher baseline UCT scores compared to other groups (33.3% vs. 62.8%; p = 0.01 and 8 vs. 5; p = 0.017). Non-responders to both drugs had an increased frequency in the female gender and lower baseline UCT scores compared to other groups (87.5% vs. 61.6%; p = 0.017 and 5 vs. 7; p = 0.06). Study Limitations: Retrospective nature, limited number of patients, no control group, the lack of the basophil activation (BAT) or BHRA (basophil histamine release assay) tests. Conclusions: Baseline disease activity assessment, which considers the presence of angioedema and disease activity scores, gender, and CRP levels might be helpful to predict treatment outcomes in CSU patients and to choose the right treatment for each patient. Categorizing patients into particular endotypes could provide treatment optimization and increase treatment success