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Introduction: Biomarkers that could reliably anticipate the effectiveness of antihistamines and omalizumab in treating chronic spontaneous urticaria (CSU) have not been conclusively identified. Our objective was to examine how eosinophilic cationic protein (ECP), tryptase, D-dimer, and total Immunoglobulin E (IgE) impact the response to antihistamine and omalizumab treatments in individuals with CSU. Methods: In this cross-sectional retrospective study, CSU patients that had undergone treatment with either antihistamines or omalizumab for a minimum of 12 weeks between 2015 and 2021 at an Allergy and Immunology Department were analyzed. Several demographic and laboratory parameters including eosinophil counts, mean platelet volüme (MPV), sedimentation, C-reactive protein (CRP), antinuclear antibodies (ANA) and Anti-thyroperoxidase (Anti-TPO) and total IgE, tryptase, ECP and D-dimer were retrived from patient files. The association of these biomarkers with Urticaria Control Test (UCT) and the effect of these biomarkers on treatment response were evaluated. Treatment response was assessed using the UCT, with a score of UCT ≥ 12 indicating a responder and UCT

Abstract Background Determination of control level in recurrent angioedema (RAE) is necessary to guide management. Here, we validated a Turkish version of the angioedema control test (AECT) for 4‐week (AECT‐4wk) and for 3‐month (AECT‐3mth) and assessed their utility in monitoring RAE. Method The recommended structured translation process for patient‐reported outcome measures was completed. The final versions were administered to 51 patients with mast cell‐mediated angioedema (MMAE) and 38 patients with hereditary angioedema, and the minimal clinically important difference (MCID) was determined. Additionally, anchor surveys comprising angioedema activity score for 28 days (AAS‐28 day), visual analog score for angioedema control, Likert scale for the control level from the patient's perspective (LS‐AEC), angioedema quality of life, short form‐12 (SF‐12) and patients' assessment of treatment sufficiency were applied. Results The Turkish AECT versions showed good convergent validity with a substantial correlation with anchor tools and known‐group validity. Excellent internal consistency and reproducibility were observed. Equal or more than 10 of 16 points scored with the AECT‐4wk and AECT‐3mth identified patients with well‐controlled disease. The disease activity, control and burden parameters were consistent with the disease control level defined depending on the cut‐off point 10 of AECT. Three‐point changes in AECT‐4wk and ‐3 mt could detect MCID in disease control in all patients. Conclusions Turkish AECT versions are valid and reliable tools for assessing and monitoring disease control in patients with RAE. The use of the Turkish versions of the AECT in routine patient care, clinical trials and angioedema research is recommended.

Abstract Background Although there have been significant advances in the treatment of chronic spontaneous urticaria (CSU) in recent years, there remains a lack of clear guidance on when and how to step down treatment in responders. This study aims to investigate stepping down approaches of different steps of CSU treatment from a global perspective. Methods “Stepping down chronic spontaneous urticaria treatment” (SDown‐CSU) is an international, multicenter, observational, cross‐sectional, survey‐based study of the Urticaria Centers of Reference and Excellence (UCARE) network. The questionnaire included 48 questions completed by physicians in the UCARE network. Results Surveys completed by 103 physicians from 81 UCAREs and 34 countries were analyzed. Seventy‐eight percent of the participants responded that they had a national urticaria management guideline written by their professional societies and 28% responded that they had to operate under a regulatory guideline proposed by central health funding organizations. Seventy‐two and 58.7% of these national recommendations do not contain any detailed information on when and/or how CSU treatment should be discontinued. There was a lack of detailed information on antihistamines and cyclosporine in particular. A predefined maximum duration was generally not applicable to omalizumab and cyclosporine (81% and 82%, respectively). Nearly all UCAREs step down omalizumab within 6 months from the first controlled status and 42% discontinue cyclosporine after 6 months regardless of the control status. Conclusions The findings from the SDown‐CSU study clearly highlight a global need for guidance on the process of stepping down treatment in CSU. Additionally, the study offers a step‐down algorithm applicable to all stages of CSU treatment.

The signs and symptoms of chronic urticaria (CU) are caused by the activation and degranulation of skin mast cells (MCs). Recent studies have added to our understanding of how and why skin MCs are involved and different in CU. Also, novel and relevant mechanisms of MC activation in CU have been identified and characterized. Finally, the use of MC-targeted and MC mediator-specific treatments has helped to better define the role of the skin environment, the contribution of specific MC mediators, and the relevance of MC crosstalk with other cells in the pathogenesis of CU. Here, we review these recent findings and their impact on our understanding of CU, with a focus on chronic spontaneous urticaria (CSU). Also, we highlight open questions, issues of controversy, and unmet needs, and we suggest what studies should be performed moving forward.

Abstract Background Urticarial vasculitis (UV) should be differentiated from chronic spontaneous urticaria (CSU) in patients initially presenting with recurrent wheals, although criteria for differential diagnosis remain ill‐defined. Objectives To set the goals, define criteria and unmet needs in UV diagnosis and differential diagnosis with CSU, and explore the possibility of coexistence of both diseases. Methods Thirteen experts experienced in UV research participated in a Delphi survey of European Academy of Allergy and Clinical Immunology taskforce. This Delphi survey involved three rounds of anonymous responses to n = 32 questions with the aim to aggregate the experts' opinions and to achieve consensus. Urticaria specialists (n = 130, most from Urticaria Centers of Reference and Excellence) evaluated the consensus statements and recommendations in the fourth and final round. Results The panel agreed that essential criteria to guide a skin biopsy in patients with recurrent wheals should include at least one of the following features: wheal duration >24 h, bruising/postinflammatory hyperpigmentation, and systemic symptoms. Leukocytoclasia and fibrin deposits were identified as a minimum set of UV histological criteria. As agreed by the panel members, CSU and normocomplementemic UV (NUV) may coexist in some patients. Conclusions The use of established criteria for the diagnosis and differential diagnosis of UV in patients with recurrent wheals can help guide the diagnostic approach and prompt earlier treatment. Further studies should investigate whether CSU and NUV are different entities or part of a disease spectrum.

Abstract Introduction The integrated care pathways for atopic dermatitis (AD‐ICPs) aim to bridge the gap between existing AD treatment evidence‐based guidelines and expert opinion based on daily practice by offering a structured multidisciplinary plan for patient management of AD. ICPs have the potential to enhance guideline recommendations by combining interventions and aspects from different guidelines, integrating quality assurance, and describing co‐ordination of care. Most importantly, patients can enter the ICPs at any level depending on AD severity, resources available in their country, and economic factors such as differences in insurance reimbursement systems. Methods The GA2LEN ADCARE network and partners as well as all stakeholders, abbreviated as the AD‐ICPs working group, were involved in the discussion and preparation of the AD ICPs during a series of subgroup workshops and meetings in years 2020 and 2021, after which the document was circulated within all GAL2EN ADCARE centres. Results The AD‐ICPs outline the diagnostic procedures, possible co‐morbidities, different available treatment options including differential approaches for the pediatric population, and the role of the pharmacists and other stakeholders, as well as remaining unmet needs in the management of AD. Conclusion The AD‐ICPs provide a multidisciplinary plan for improved diagnosis, treatment, and patient feedback in AD management, as well as addressing critical unmet needs, including improved access to care, training specialists, implementation of educational programs, assessment on the impact of climate change, and fostering a personalised treatment approach. By focusing on these key areas, the initiative aims to pave the way for a brighter future in the management of AD.

Abstract 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. © 2022 Published by Elsevier España, S.L.U. on behalf of Sociedade Brasileira de Dermatologia. This is an open access article under the CC BY license (https://creativecommons.org/licenses/by/4.0/).

Since more than a century ago, there has been awareness of the connection between viral infections and the onset and exacerbation of urticaria. Our knowledge about the role of viral infection and vaccination in acute and chronic urticaria improved as a result of the COVID-19 pandemic but it has also highlighted knowledge gaps. Viral infections, especially respiratory tract infections like COVID-19, can trigger the onset of acute urticaria (AU) and the exacerbation of chronic urticaria (CU). Less frequently, vaccination against viruses including SARS-CoV-2 can also lead to new onset urticaria as well as worsening of CU in minority. Here, with a particular focus on COVID-19, we review what is known about the role of viral infections and vaccinations as triggers and causes of acute and chronic urticaria. We also discuss possible mechanistic pathways and outline the unmet needs in our knowledge. Although the underlying mechanisms are not clearly understood, it is believed that viral signals, medications, and stress can activate skin mast cells (MCs). Further studies are needed to fully understand the relevance of viral infections and vaccinations in acute and chronic urticaria and to better clarify causal pathways.

Chronic urticaria (CU) is a mast cell-driven chronic inflammatory disease with a female predominance. Since CU affects mostly females in reproductive age, pregnancy is an important aspect to consider in the context of this disease. Sex hormones affect mast cell (MC) biology, and the hormonal changes that come with pregnancy can modulate the course of chronic inflammatory conditions, and they often do. Also, pregnancy-associated changes in the immune system, including local adaptation of innate and adaptive immune responses and skewing of adaptive immunity toward a Th2/Treg profile have been linked to changes in the course of inflammatory diseases. As of now, little is known about the effects of pregnancy on CU and the outcomes of pregnancy in CU patients. Also, there are no real-life studies to show the safety of urticaria medications during pregnancy. The recent PREG-CU study provided the first insights on this and showed that CU improves during pregnancy in half of the patients, whereas it worsens in one-third; and two of five CU patients experience flare-ups of their CU during pregnancy. The international EAACI/GA2LEN/EuroGuiDerm/APAAACI guideline for urticaria recommends adopting the same management strategy in pregnant and lactating CU patients; starting treatment with standard doses of second-generation (non-sedative) H1 antihistamines, to increase the dose up to 4-folds in case of no response, and to add omalizumab in antihistamine-refractory patients; but also emphasizes the lack of evidence-based information on the safety and efficacy of urticaria treatments during pregnancy. The PREG-CU study assessed treatments and their outcomes during pregnancy. Here, we review the reported effects of sex hormones and pregnancy-specific immunological changes on urticaria, we discuss the impact of pregnancy on urticaria, and we provide information and guidance on the management of urticaria during pregnancy and lactation.

Abstract Background Information/communication technologies such as mobile phone applications (apps) would enable chronic urticaria (CU) patients to self‐evaluate their disease activity and control. Yet, recently Antó et al (2021) reported a global paucity of such apps for patients with CU. In this analysis, we assessed patient interest in using apps to monitor CU disease activity and control using questions from the chronic urticaria information and communication technologies (CURICT) study. Methods The methodology for CURICT has been reported. Briefly, a 23‐item questionnaire was completed by 1841 CU patients from 17 UCAREs across 17 countries. Here, we analyzed patient responses to the CURICT questions on the use of apps for urticaria‐related purposes. Results As previously published, the majority of respondents had chronic spontaneous urticaria (CSU; 63%; 18% chronic inducible urticaria (CIndU) [CIndu]; 19% with both), were female (70%) and in urban areas (75%). Over half of patients were very/extremely interested in an app to monitor disease activity (51%) and control (53%), while only ∼1/10 were not. Patients with both urticaria types versus those with CSU only (odds ratio [OR], 1.36 [1.03–1.79]) and females versus males (OR [95% CI], 1.47 [1.17–1.85]) were more likely to be very to extremely interested in an app to assess disease control. Conclusions Overall, half of the patients with CU were very to extremely interested in using an app to assess their disease activity and control. Development of well‐designed apps, specific to disease types (CSU, CIndU, CSU + CIndU, etc), validated by experts across platforms would help improve the management and possibly outcomes of CU treatment while providing important patient information to be used in future research.

Abstract Chronic urticaria is a disease characterized by the appearance of weals, angioedema or both longer than 6 weeks. Degranulation of cutaneous or submucosal mast cells leads to release of mediators including histamine resulting in redness, swelling and itch. Because mast cells are widely distributed throughout the body, the question is why they are not activated systemically or does systemic activation occur without overt end organ dysfunction? We have conducted an exploratory literature search for reports that have evidence of organ-specific dysfunction in chronic urticaria that might justify prospective observational studies. This search revealed some evidence of systemic effects of chronic urticaria in cardiac, respiratory, gastrointestinal, central nervous and musculo-skeletal systems. The relevance of these findings needs to be further determined. However, they justify prospective studies in larger numbers of patients and at different stages of disease activity.

Background: Patients with chronic urticaria (CU) are increasingly using information and communication technologies (ICTs) to manage their health. What CU patients expect from ICTs and which ICTs they prefer remains unknown. We assessed why CU patients use ICTs, which ones they prefer, and what drives their expectations and choices. Methods: In this cross-sectional study, 1841 patients across 17 countries were recruited at UCAREs (Urticaria Centers of Reference and Excellence). Patients with CU who were >12 years old completed a 23-item questionnaire. Results: Most patients were interested in receiving disease information (87.3%), asking physicians about CU (84.1%), and communicating with other patients through ICTs (65.6%). For receiving disease information, patients preferred one-to-one and one-to-many ICTs, especially web browsers. One-to-one ICTs were also the ICTs of choice for asking physicians about urticaria and for communicating with other patients, and e-mail and WhatsApp were the preferred ICTs, respectively. Many-to-many ICTs such as Facebook, Instagram, LinkedIn, and Twitter were least preferred for all 3 purposes. Living in rural areas and higher education were linked to higher odds of being interested in receiving disease information, asking physicians, and communicating with patients through ICTs. Conclusions: Most patients and especially patients with higher education who live in rural areas are interested in using ICTs for their healthcare, but prefer different ICTs for different purposes, ie, web browsers for obtaining information, e-mail for asking physicians, and WhatsApp for communicating with other patients. Our findings may help to improve ICTs for CU.

Background: Patients with chronic spontaneous urticaria often exhibit psychiatric comorbidities including depression that contribute to the impairment of their quality of life. How CSU and depression are linked isn't well-understood. Substance P has been shown to be increased in patients with CSU and is held to contribute to the pathogenesis of depression.Methods: We measured disease activity in 30 CSU patients without depression and 30 CSU patients with depression by using the urticaria activity score. The severity of depression was assessed with the Beck Depression Inventory. We measured SP levels in these patients as well as in 30 healthy control subjects. In patients with comorbid depression, we correlated SP levels with CSU disease activity and the severity of depression.Results: In CSU patients, disease activity and the severity of depression were positively linked. UAS7 values were higher in CSU patients with comorbid depression as compared to those without (p < 0.05). SP levels were higher in CSU patients with depression than in those without (p < 0.001), but was similar in all CSU patients compared to healthy controls. SP levels weren't correlated with UAS7 values in CSU patients with depression, whereas they were weakly but significantly correlated with BDI scores (p < 0.05).Conclusion: Our results suggest that, in CSU patients with comorbid depression, CSU disease activity affects the severity of depression. CSU patients with high disease activity should be explored for comorbid depression.

Background: Chronic urticaria (CU) is characterized by itchy recurrent wheals, angioedema, or both for 6 weeks or longer. CU can greatly impact patients' physical and emotional quality of life. Patients with chronic conditions are increasingly seeking information from information and communications technologies (ICTs) to manage their health. The objective of this study was to assess the frequency of usage and preference of ICTs from the perspective of patients with CU. Methods: In this cross-sectional study, 1800 patients were recruited from primary healthcare centers, university hospitals or specialized clinics that form part of the UCARE (Urticaria Centers of Reference and Excellence) network throughout 16 countries. Patients were >12 years old and had physician-diagnosed chronic spontaneous urticaria (CSU) or chronic inducible urticaria (CIndU). Patients completed a 23-item questionnaire containing questions about ICT usage, including the type, frequency, preference, and quality, answers to which were recorded in a standardized database at each center. For analysis, ICTs were categorized into 3 groups as follows: one-to-one: SMS, WhatsApp, Skype, and email; one-to-many: YouTube, web browsers, and blogs or forums; many-to-many: Instagram, Twitter, Facebook, and LinkedIn. Results: Overall, 99.6% of CU patients had access to ICT platforms and 96.7% had internet access. Daily, 85.4% patients used one-to-one ICT platforms most often, followed by one-to-many ICTs (75.5%) and many-to-many ICTs (59.2%). The daily ICT usage was highest for web browsers (72.7%) and WhatsApp (70.0%). The general usage of ICT platforms increased in patients with higher levels of education. One-to-many was the preferred ICT category for obtaining general health information (78.3%) and for CU-related information (75.4%). A web browser (77.6%) was by far the most commonly used ICT to obtain general health information, followed by YouTube (25.8%) and Facebook (16.3%). Similarly, for CU-specific information, 3 out of 4 patients (74.6%) used a web browser, 20.9% used YouTube, and 13.6% used Facebook. One in 5 (21.6%) patients did not use any form of ICT for obtaining information on CU. The quality of the information obtained from one-to-many ICTs was rated much more often as very interesting and of good quality for general health information (53.5%) and CU-related information (51.5%) as compared to the other categories. Conclusions: Usage of ICTs for health and CU-specific information is extremely high in all countries analyzed, with web browsers being the preferred ICT platform.

Background: Urticarial vasculitis (UV) is a rare type of leukocytoclastic vasculitis characterized by long lasting urticarial skin lesions and poor response to treatment. As of yet, no clinical guidelines, diagnostic criteria, or treatment algorithms exist, and the approaches to the diagnostic workup and treatment of UV patients may differ globally. We conducted an online survey to examine how UV patients are diagnosed and treated by international specialists and to reveal the greatest challenges in managing UV patients worldwide. Methods: Distribution of the questionnaire included an email to individuals in the World Allergy Organization (WAO) database, with no restrictions applied to the specialty, affiliation, or nationality of the participants (November 2018). The email contained a link (Internet address) to the online questionnaire. Responses were anonymous. The link to the questionnaire was further sent to the network of Urticaria Centers of Reference and Excellence (UCARE) in the Global Allergy and Asthma European Network (GA2LEN) as well as to the Turkish Dermatology Society and the Japanese Society of Allergology, who distributed the link to their members. In addition, the survey link was posted online in the group of the Russian Society of Allergologists and Immunologists. Results: We received 883 completed surveys from physicians in 92 countries. UV was reported to be rare in clinical practice, with an average of 5 patients per physician per year. More than two-thirds of physicians reported wheals, burning of the skin, and residual hyperpigmentation in 60–100% of UV patients. The most frequently reported reason for receiving referrals of patients with UV was to establish the diagnosis. The most important features for establishing the diagnosis of UV were wheals of longer than 24 hours duration (72%), the results of skin biopsy (63%), and post-inflammatory hyperpigmentation (46%). The most common tests ordered in UV patients were complete blood count, erythrocyte sedimentation rate, C-reactive protein, complement components, antinuclear antibodies, and skin biopsy. Physicians considered UV to be of unknown cause in most patients, and drugs and systemic lupus erythematosus to be the most common identifiable causes. Two of 3 physicians reported that they use second-generation antihistamines in standard dose as the first-line therapy in patients with UV. The greatest perceived challenges in the management of UV were the limited efficacy of drugs and the absence of clinical guidelines and treatment algorithms. Conclusions: UV is a challenging disease. Skin biopsy, a gold standard for UV diagnosis, is not performed by many physicians. This may lead to misdiagnosis of UV, for example, as chronic spontaneous urticaria, and to inadequate treatment. International consensus-based recommendations for the classification of UV and the diagnostic workup and treatment, as well as prospective studies evaluating potentially safe and effective drugs for the treatment of UV, are necessary. Keywords: Urticarial vasculitis, Management, Worldwide, Treatment, Diagnosis

Background/Objectives: Chronic spontaneous urticaria (CSU) is a common skin disorder which represents a challenge both for the patients and physicians. Guidelines and treatment algorithms have been created to help physicians to ease management. Our aim was to determine Turkish dermatologists' approach to CSU with regard to treatment, search for causative factors and use of instruments to assess the quality of life and severity of the disease. Methods: This was a cross-sectional methodological study which was performed by delivery of a questionnaire including ten questions about the management of CSU. Results: Analyses of 314 questionnaires revealed that the most common first-line treatments were non-sedating antihistamines in standard doses (65.6%), while second-line treatment was updosing antihistamines (59.9%) followed by addition of sedative-antihistamines (26.4%) and systemic steroids (19.1%). Third-line treatment option was omalizumab in 35% followed by systemic steroids. Twenty-two percent of the dermatologists referred the patients to a center experienced in urticaria. Most of them were performing laboratory testing for underlying causes including thyroid function tests, C-reactive protein, thyroid auto-antibodies, stool analyses, infection markers. Urticaria activity score and chronic urticaria quality of life questionnaire were used by 30 and 13%, respectively, while 56% were using none of the instruments. Conclusion: Our study showed that the therapeutic management of Turkish dermatologists was parallel to the European Urticaria Guidelines. The high utility of omalizumab as a third line regimen improved patient care. Nevertheless there is a need for centers experienced in urticaria to refer antihistamine-resistant patients where third-line treatment options can not be implemented. Keywords: Urticaria, Dermatologists, Therapeutics, Diagnosis, Guideline

Neutrophilic dermatoses comprise a heterogeneous group of disorders, which are characterized by inflammatory skin lesions that share a common histopathological feature, intense inflammatory infiltration consisting primarily of neutrophils, with no evidence of vasculitis. We describe a 75-year-old man with rheumatoid arthritis, who presented with erythematous edematous plaques. The histopathological findings indicated neutrophilic dermatosis; however, the patient was afebrile and without an elevated white blood cell count or systemic involvement. We think that the most suitable expression for this variant with clinical manifestations different from those of classical Sweet syndrome is “chronic recurrent annular neutrophilic dermatosis,” as introduced by Christensen et al.1

Background: The urticaria control test (UCT) is a questionnaire designed to determine if chronic urticaria (CU) is controlled or not and to aid therapeutic decision-making. It collects retrospective information about the symptoms and quality of life impairment over the last 4 weeks. The current study aimed to investigate the validity, reliability and sensitivity to change of the Turkish version of the UCT. We also evaluated its correlation with other tools and compared the UCT results of patients with chronic spontaneous urticaria (CSU) and patients with chronic inducible urticaria (CINDU). Methods: Following forward/backward translation and cognitive debriefing, the Turkish version of the UCT was used in 81 CSU and 78 CINDU patients. Dermatology life quality index (DLQI), Chronic urticaria quality of life questionnaire (CU-Q2oL), urticaria activity score (UAS), patients' and physicians’ global assessment visual analog scores and Likert scales were used at baseline and after four weeks to assess quality of life impairment, disease activity and disease control. Statistical analysis to determine the validity and reliability of the Turkish version of the UCT as well as comparison between CINDU and CSU patients were performed. Results: Duration of disease was longer, disease control was poorer and severe complaints were more frequent in CINDU patients (duration of disease: 36.3 (24) ± 49.1 vs 31.5 (9) ± 67.9, p = .007, UCT baseline: 8.4 (8) ± 3.4 vs 10.4 (11) ± 3.9, p = .001 and patient's global assessment Likert scale severe complaints: 6 vs 15, p

Pruritus is the most frequently described symptom in dermatology and can significantly impair the patient’s quality of life. In 10–50% of adults with persistent pruritus, it can be an important dermatologic clue for the presence of a significant underlying systemic disease such as renal insufficiency, cholestasis, hematologic disorder, or malignancy (Etter and Myers, 2002; Zirwas and Seraly, 2001). This review describes the presence of pruritus in different systemic diseases. It is quite important to discover the cause of pruritus for providing relief for the patients experiencing substantial morbidity caused by this condition.

Acute generalized exanthematous pustulosis (AGEP) is a skin eruption that is primarily drug induced and characterized by the formation of numerous acute and sterile pustules on an erythematous background as mentioned by Weinblatt et al. (1999). We present a case of AGEP, following administration of etanercept, an antitumour necrosis factor alpha (TNF-α) antibody, in a patient with psoriasis. Recognition of this reaction pattern is important given the frequent reliance on etanercept in treating psoriasis.

Background: Altered microbiota has been implicated in the pathogenesis of chronic spontaneous urticaria (CSU). However, studies showing the effects of probiotic supplementation in CSU are scarce. Objectives: The objective of the study was to evaluate the efficacy of oral probiotic supplementation (Lactobacillus reuteri) in patients with CSU. Methods: This was a prospective, nonrandomized, parallel group, and controlled study. Oral probiotic supplementation (L. reuteri ATCC 55730 1 × 108 CFU) and a single dose of ebastine were introduced to 30 CSU patients, whereas a control group of 30 CSU patients received only a single dose of ebastine. Treatment responses, symptom control, and quality of life (QoL) were evaluated with Urticaria Activity Score 7 (UAS7), Physicians’ Global Assessment Visual Analog Score (PhyGA-VAS), and Chronic Urticaria Quality of Life Questionnaire (CU-Q2oL) at weeks 0, 2, and 4. Results: There were significant decreases in the UAS7 and CU-Q2oL and increases in PhyGA-VAS both in the probiotic group and control group at weeks 2 and 4 (P < 0.05; for all). The changes in the UAS7, CU-Q2oL, and PhyGA-VAS were significantly higher in the probiotic group than in the control group (P = 0.026; P = 0.001; and P = 0.004). The number of responders was significantly higher in the probiotic group than in the control group (83.3% vs. 50%, P = 0.006). Complete response and significant clinical improvement were observed in 9 (30%) and 20 (66.7%) patients in the probiotic group and 6 (20%) and 12 (40%) patients in the control group, respectively. High levels of total immunoglobulin E (≥100 IU/mL) were more frequent in probiotic nonresponders than in probiotic responders. Conclusion: Probiotics, when added to the standard therapy may improve the symptoms as well as the QoL scores in the treatment of CSU.

Urticaria is a common disease that can affect individuals of all age groups, with approximately one-quarter of the population experiencing it at least once in their lifetime. Lesions characterized by erythema and itchy hives can appear anywhere on the body. These can vary in size ranging from millimeters to centimeters, and typically clear within 24 h. About 40% of patients with urticaria have accompanying angioedema, which involves localized deep tissue swelling. Urticaria usually occurs spontaneously and is classified into acute and chronic forms, with the latter referring to a condition that lasts for more than 6 weeks. The prevalence of chronic urticaria in the general population ranges from 0.5% to 5%, and it can either be inducible or spontaneous. The most common form of pediatric urticaria is acute and is usually self-limiting. However, a broad differential diagnosis should be considered in children with urticaria, particularly if they also have accompanying systemic complaints. Differential diagnoses of pediatric urticaria include chronic spontaneous urticaria, chronic inducible urticaria, serum sickness-like reaction, urticarial vasculitis, and mast cell disorders. Conditions that can mimic urticaria, including but not limited to cryopyrinopathies, hyper IgD syndrome, Periodic Fever, Aphthous Stomatitis, Pharyngitis and Adenitis (PFAPA), Tumor Necrosis Factor Receptor Associated Periodic Syndrome (TRAPs), and Schnitzler syndrome should also be considered. The many faces of pediatric urticaria can be both easy and confusing. A pragmatic approach relies on clinical foresight and understanding the various forms of urticaria and their potential mimickers. This approach can pave the way for an accurate and optimized diagnostic approach in children with urticaria.

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Omalizumab is a humanized monoclonal anti-immunoglobulin E which is originally developed for the treatment of allergic respiratory disorders, but has been approved for the treatment of antihistamine-resistant chronic spontaneous urticaria in 2014. It has become a game changer in the treatment of all subtypes of chronic urticaria (CU) even though it has not been yet approved for inducible urticarias. It is a safe drug with minor adverse events reported in the clinical trials as well as real life studies. The main questions that remain to be answered regarding its use for CU are the duration of treatment, how to cease the treatment and safety of long-term use. Available information about these questions are tried to be provided in this review. [ABSTRACT FROM AUTHOR]

Background and Design: Albeit an easily recognized disease, urticaria features many diverse approaches which rationalize the need for an algorithm for the diagnosis, classification, etiopathogenesis, diagnostic evaluation and therapeutic approach. Therefore, authors from Dermatoallergy Working Group of the Turkish Society of Dermatology and the Turkish Dermatoimmunology and Allergy Association aimed to create an urticaria guideline for the diagnosis, treatment and follow- up of urticaria. Materials and Methods: Each section of the guideline has been written by a different author. The prepared sections were evaluated in part by e-mail correspondence and have taken its final form after revision in the last meeting held by the participation of all authors. Results: The guideline includes the description, classification, pathophysiology as well as diagnosis and treatment of urticaria. Urticaria is classified into two main types: acute urticaria and chronic urticaria while chronic urticaria is further subdivided into spontaneous urticaria and inducible urticaria. The first step of treatment includes standard doses of H1-blockers. In patients who do not respond to the first step, antihistamine dose is increased up to four times; if unsuccessful, another second-generation antihistamine is given in the same dose. In antihistamine-resistant cases, introduction of omalizumab is required. Omalizumab dose may be increased in patients failing to respond to the standard dose. In patients unresponsive to omalizumab, cyclosporine-A may be given. Routine diagnostic tests are not recommended in acute urticaria. In chronic urticaria, erythrocyte sedimentation rate, differential blood count and C-reactive protein testing are the only investigations that are needed routinely. Conclusion: Chronic urticaria is a disease that can be challenging for the physician in terms of treatment and follow-up. Depending on evidence-based data (and individual experiences), this guideline will have a leading role in the diagnosis and treatment of urticaria and help the physician to overcome the challenges in the management. [ABSTRACT FROM AUTHOR]

Objective: Recurrent Angioedema (RAE) is characterized by sudden swelling of mucosal surfaces or deep dermis and is either mast cell-(MMAE) or bradykinin-mediated (BMAE). How patients with BMAE and MMAE differ in terms of disease activity and impact remains largely unknown. Here, we determined validity, reliability, and sensitivity to change of Turkish versions of angioedema activity score (AAS) and quality of life questionnaire (AE-QoL) and used both instruments to investigate and compare patients with BMAE and MMAE. Methods: Turkish versions of AAS28 and AE-QoL were applied to 94 patients with RAE (18–72 years). Patients’ global self-assessment of QoL (PGA-QoL), disease activity (PGA-DA-VRS, PatGA-DA-VAS), and 12-Item-Short Form Survey were used at week 4 (visit 2), and week 8 (visit 3). Demographic characteristics, clinical features, and AAS28 and AE-QoL values were compared between 31 patients with BMAE and 63 patients with MMAE. Results: Turkish AAS28 and AE-QoL showed excellent internal consistency, high reproducibility and known-groups validity. Compared to patients with MMAE, BMAE patients were younger (34.6 ± 10.7 vs. 40.7 ± 13.3 years), had longer disease duration (236 ± 178 vs. 51 ± 78 months), high prevalence of family history (63% vs 14%), longer duration of attacks (65 ± 20 vs. 40 ± 25 h), and they were more commonly affected by upper airway angioedema (70% vs 23%). Disease activity (AAS28) was lower (29.3 ± 24.6 vs 55.2 ± 52.9), but AE-QoL was higher (44.2 ± 16.1 vs 34.5 ± 22.5) in BMAE patients as compared to MMAE patients. Conclusions: Patients with BMAE and MMAE have distinct disease characteristics. Recurrent bradykinin-mediated angioedema impacts quality of life more than mast cell-mediated angioedema. The discriminating characteristics of patients with BMAE and MMAE may help to improve the diagnosis and management of patients with RAE.

Background and Design: Occupational hand eczema is a commonly encountered problem. The time interval between onset and diagnosis is often long and this leads to increased morbidity. Besides dermatologists, family practitioners and workplace doctors play a role in the diagnosis and treatment of the disease. We aimed in our study to evaluate the discrepancies in how the disease is diagnosed and how the patients are handled between these specialty groups. We also aimed to determine the level of knowledge about occupational eczema and how the knowledge could be increased. Materials and Methods: This was a descriptive study performed between January 2012 and December 2013. A questionnaire was sent via email to dermatologists, family practitioners and workplace doctors. Results: A total of 393 doctors, including 313 dermatologists, 53 family practitioners and 27 workplace doctors were enrolled in the study. Sixty-three percent of the doctors were examining 10-100 patients with hand eczema in a mouth. Eighty-five percent was "always" asking the occupation of the patient. 71.7% was asking "everytime" what materials are handled in the work place. The answers to the question "What is your approach to the patient after you diagnose occupational hand eczema" was "I refer the patient" and "I tell the patient to quit the job" was higher in family practitioners and family doctors. The center for referral was "dermatologists" for the workplace doctors and family practitioners while dermatologists referred the patients to "a clinic performing patch tests" or "occupational diseases centers". 91.2% of the responders stated that they wanted to have more knowledge about occupational eczema and the issue of wonder mostly found was "the allergens/irritants encountered in the workplace". Conclusion: A high number of the respondents stated that they wanted to gain more knowledge about occupational eczema. More courses, meetings, seminars are required to be performed on this topic and more papers or text-books concerning the subject should be published. In addition, special centers for the diagnosis, care and rehabilitation of these patients are needed. [ABSTRACT FROM AUTHOR]

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Purpose: To describe the ocular angiographic findings in patients with Neurofibromatosis type 1 (NF-1). Methods: We examined 20 consecutive patients diagnosed with NF1 by conventional ophthalmoscopy and fluorescein angiography (FA), indocyanine green angiograms (ICGA) were also obtained from 11 of the patients. Forty FAs and 25 ICGAs were also obtained from age-matched controls. Results: Fluorescein angiography revealed patchy hyperfluorescence in 30%, retinal pigment epithelial (RPE) atrophy in 30%, “corkscrew” vessels in 50%, optic disc abnormalities in 25% and choroidal nevi in 30% of the patients. All the patients investigated by ICGA (n = 11) demonstrated hypofluorescent lesions corresponding to the patchy hyperfluorescence on fluorescein angiography. None of the control patients had any of the findings described neither on FA nor on ICGA. Conclusion: Because of their high frequency (100%), the hypofluorescent lesions on ICGA should be added as a new diagnostic criterion in NF1. RPE atrophy and the high frequency of optic disc abnormalities appear to be novel findings in patients with NF-1. [ABSTRACT FROM AUTHOR]

Background/Objectives: Dermatological diseases not only affect patients' lives but also the lives of their family members. Our aim was to evaluate anxiety and depression risk in patients with acne vulgaris and the effect of acne vulgaris on quality of life of the patients and their families. Methods: A total of 125 patients with acne vulgaris, 110 of their family members, and 100 healthy volunteers were included in the study. Patients with acne vulgaris were requested to complete the Acne Quality of Life Scale (AQOL) and Hospital Anxiety and Depression Scale (HAD) at the time of the first admission and 2 months later. The healthy volunteers were only asked to complete the HAD, and the family members filled out the Family Dermatology Life Quality Index (FDLQI) at the time of the first admission and 2 months later. Results: The acne and control groups showed no significant differences between the HAD anxiety subscale (HAD-A) and HAD depression subscale (HAD-D) scores. The mean AQOL score of the patients was 6.8 ± 5.3. AQOL scores were positively correlated with HAD-A, HAD-D and FDLQI scores. The mean score of the FDLQI was 7.6 ± 5.3. FDLQI scores showed a statistically significant change after 2 months (p = 0.001). Conclusion: Acne vulgaris does not have an effect on quality of life and the risk of anxiety or depression. In the cases of acne, when the quality of life decreases, the risk of depression as well as anxiety increases and the quality of life of the family members is negatively affected. Acne vulgaris negatively affects the quality of life of the family members of the patients.

Abstract Acute generalized exanthematous pustulosis is a rare drug-induced eruption that is characterized by acute, nonfollicular sterile pustules on an erythematous and edematous base. The most frequently implicated drugs are beta-lactam antibiotics. Hydroxychloroquine has been widely used to treat dermatologic and rheumatologic diseases and has been reported as a rare cause of acute generalized exanthematous pustulosis. A 42-year-old female presented with pustular lesions on the skin surface with erythema, facial edema, and occasional atypical target-like lesions after 21 days of treatment with 200mg/day hydroxychloroquine for rheumatoid arthritis, diagnosed one month previously. We report a case with acute generalized exanthematous pustulosis induced by hydroxychloroquine and treated with dapsone and systemic corticosteroid.

Bullae occurring in lesions of morphea are uncommon. The cause of bullae formation in morphea is multifactorial, although lymphatic obstruction from the sclerodermatous process is considered the likeliest cause. Bullous morphea may be confused clinically with lichen sclerosus et atrophicus since both diseases may cause bullae in sclerodermatous plaques. A 69-year-old woman presented with a history of generalized morphea diagnosed 9 years earlier; and a 1-month history of pruritic bullae on her inframammary folds, axillary regions, lower abdomen, upper extremities and inguinal folds. Physical examination revealed multiple erythematous erosions, hemorrhagic vesicles and eroded bullae with slight scale or crusts overlying hypopigmented, indurated, shiny plaques. Skin biopsy revealed prominent edema in the papillary dermis, resulting in bulla formation and thickening of collagen fibers within the dermis. Direct immunofluorescence was negative. According to histologic and clinical features, the diagnosis of bullous morphea was established. [ABSTRACT FROM AUTHOR]

Abstract Inducible urticaria is a heterogeneous subgroup of chronic urticarias caused by a wide variety of environmental stimuli, such as exercise, cold, heat, pressure, sunlight, vibration, and water. A new term, follicular traction urticaria, was suggested as an unusual form of inducible urticarias. We report a patient who was diagnosed with follicular traction urticaria.

Background. Psoriasis is a chronic inflammatory skin disorder in which proinflammatory cytokines including IL-6 and TNF-α increase both locally and systematically. It is thought that chronic inflammation results in metabolic diseases and proinflammatory cytokines give rise to the development of atherogenesis, peripheral insulin resistance, hypertension, and type 2 diabetes. Our aim was to investigate the prevalence of metabolic syndrome in patients with psoriasis vulgaris. Methods. Study consisted of 115 plaque-type psoriasis patients and 140 healthy individuals. Data including body weight, height, waist circumference, body-mass index, and arterial blood pressure were collected. Fasting blood glucose, triglyceride, and HDL levels were determined. International Diabetes Federation Criteria for Metabolic Syndrome and Insulin Resistance were used for evaluating patients with metabolic syndrome and diabetes. Results. Compared to the control group, metabolic syndrome, diabetes mellitus, and hypertension were found to be higher in psoriasis patients. Metabolic syndrome was increased by 3-folds in psoriasis patients and was more prevalent in women than in men. It was determined that the prevalence of metabolic syndrome was higher in psoriasis patients after the age of 40. Metabolic syndrome was not related to smoking, severity of psoriasis, and duration of disease. Conclusions. Our findings suggest that psoriasis preconditions occurrence of a group of diseases such as diabetes mellitus, hypertension, and metabolic syndrome. For this reason, patients with psoriasis should be treated early and they should be followed with respect to metabolic diseases.

Background. Thyroid disorders may affect all of the organ systems of the body and they are also highly associated with a wide variety of skin disorders. The aim of this study was to investigate the prevalence of thyroid function abnormalities and thyroid autoimmunity in patients with pemphigus vulgaris (PV) and to determine the association between thyroid disorders and clinical involvement and systemic corticosteroid treatment in patients with PV. Methods. The study consisted of eighty patients with PV and eighty healthy individuals. Thyroid functions (fT3, fT4, and TSH) and thyroid autoimmunity (anti-thyroid peroxidase (anti-TPO), and anti-thyroglobulin (anti-Tg) antibodies) were investigated in both groups. Primary thyroid disease (PTD) was diagnosed with one or more of the following diagnostic criteria: (i) positive antithyroid antibodies, (ii) primary thyroid function abnormalities. Results. Significant changes in the serum thyroid profile were found in 16% (13/80) of the PV group and 5% (4/80) of the control group. Positive titers of antithyroid antibodies (anti-TPO and anti-Tg) were observed in 7 patients (9%) with PV and one in the control group (1,2%). Hashimoto thyroiditis was diagnosed in 9% of PV patients and it was found to be more prevalent in the mucosal form of PV. PTD was found in 13 of (%16) PV patients which was significantly high compared to controls. PTD was not found to be associated with systemic corticosteroid use. Free T3 levels were significantly lower in PV group compared to the control group and free T4 levels were significantly higher in PV group compared to the controls. Conclusions. PV may exist together with autoimmune thyroid diseases especially Hashimoto thyroiditis and primer thyroid diseases. Laboratory work-up for thyroid function tests and thyroid autoantibodies should be performed to determine underlying thyroid diseases in patients with PV.

Prisons have been studied as communal places where risk of contagious diseases and dermatological diseases associated with stress are more frequent. We aimed to investigate the prevalence of skin diseases in female prisoners with special focus on psychological stress. We held a day-time dermatology polyclinic for 6-weeks. The patients were given Beck Depression Inventory (BDI) and a questionnaire on the psychological impact of skin disease. A total of 383 female prisoners were examined; 41 dermatological diseases were diagnosed. Acne was the most prevalent condition (34%), followed by hair loss (19%), dry skin (16%), and eczema (12%). Thirty-six percent of the prisoners felt embarrassed, 34% felt anxious, and 45% felt sad about their skin disease. Fourty seven of the responders were found to be in severe depression according to BDI responses. We could not find any association between BDI results and any kind of skin disease diagnosed in inmates. Our study demonstrates that prisoners have benign and common skin conditions similar to those in the general population.