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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., NA

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Chronic urticaria and angioedema are diseases often managed by Allergy and Immunology specialists. Recent international guidelines have outlined a stepwise approach to management of patients using dose escalation of second-generation antihistamines followed by use of omalizumab and finally cyclosporine in more refractory cases. In select patients (those with refractory chronic urticaria), nonbiologic alternative medications with anti-inflammatory or immunosuppressant activity may be considered. Angioedema without wheals may have several different pathophysiologic mechanisms. Optimal management of mast cell-mediated angioedema is less clear but is often managed similar to chronic spontaneous urticaria. Drug-induced angioedema due to angiotensin-converting enzyme inhibitors is a common cause of angioedema in the emergency department. Although bradykinin is thought to be a primary mediator for this type of angioedema, studies of targeted therapies have been generally disappointing. In contrast, several targeted therapies have been proven successful using acute and preventive approaches for management of hereditary angioedema. Further developments, including novel biologics, novel oral therapies, and gene therapy approaches, may hopefully continue to broaden therapeutic options to ensure optimal individual management of patients with hereditary angioedema.

Background: cold urticaria (ColdU), that is, the occurrence of wheals or angioedema in response to cold exposure, is classified into typical and atypical forms. The diagnosis of typical ColdU relies on whealing in response to local cold stimulation testing (CST). It can also manifest with cold-induced anaphylaxis (ColdA). We aimed to determine risk factors for ColdA in typical ColdU. Methods: an international, cross-sectional study COLD-CE was carried out at 32 urticaria centers of reference and excellence (UCAREs). Detailed history was taken and CST with an ice cube and/or TempTest(R) performed. ColdA was defined as an acute cold-induced involvement of the skin and/or visible mucosal tissue and at least one of: cardiovascular manifestations, difficulty breathing, or gastrointestinal symptoms. Results: of 551 ColdU patients, 75% (n = 412) had a positive CST and ColdA occurred in 37% (n = 151) of the latter. Cold-induced generalized wheals, angioedema, acral swelling, oropharyngeal/laryngeal symptoms, and itch of earlobes were identified as signs/symptoms of severe disease. ColdA was most commonly provoked by complete cold water immersion and ColdA caused by cold air was more common in countries with a warmer climate. Ten percent (n = 40) of typical ColdU patients had a concomitant chronic spontaneous urticaria (CSU). They had a lower frequency of ColdA than those without CSU (4% vs. 39%, p = .003). We identified the following risk factors for cardiovascular manifestations: previous systemic reaction to a Hymenoptera sting, angioedema, oropharyngeal/laryngeal symptoms, and itchy earlobes. Conclusion: coldA is common in typical ColdU. High-risk patients require education about their condition and how to use an adrenaline autoinjector., GA(2)LEN UCARE Network

Background Patch testing with the European Baseline Series (EBS) is an essential diagnostic tool for the assessment of allergic contact dermatitis. Objective The aims of the study were to describe the most common contact allergens identified with patch testing in Turkey and to establish the distribution of contact allergens among different regions in Turkey. Patients and methods Twelve centers consisting of tertiary health care institutions were included in the study. A total of 1169 patients were patch tested with the EBS from 2015 to 2017. Results A total of 596 patients (51.0%) had 1 or more positive patch test reactions to the EBS. A total of 30.2% (n = 353) of those tested had clinical relevance. Contact sensitivity was more common in young patients. The most common 10 allergens were nickel sulfate (20.4%), followed by textile dye mix (8.6%), cobalt chloride (8.3%), potassium dichromate (8.0%), p-phenylenediamine base (4.5%), balsam of Peru (4.3%), methylchloroisothiazolinone/methylisothiazolinone (3.8%), thiuram mix (3.6%), fragrance mix I (3.0%), and methylisothiazolinone (2.9%). Conclusions This study provides a comprehensive profile of patch test results with the EBS in Turkey. An ongoing decrease in contact allergy to methylchloroisothiazolinone/methylisothiazolinone and methylisothiazolinone was observed. Textile dye mix and p-phenylenediamine are beginning to pose a new risk in Turkey.

Chronic urticaria (CU) affects about 1% of the world population of all ages, mostly young and middle-aged women. It usually lasts for several years (> 1 year in 25-75% of patients) and often takes > 1 year before effective management is implemented. It presents as chronic spontaneous urticaria (CSU), chronic inducible urticaria (CIndU) or both in the same person. More than 25% of cases are resistant to H1 -antihistamines, even at higher doses, and third- and fourth-line therapies (omalizumab and ciclosporin) control the disease only in two-thirds of H1 -antihistamine-resistant patients. Here we review the impact of CU on different aspects of patients' quality of life and the burden of this chronic disease for the patient and society. CU may have a strong impact on health-related quality of life (HRQoL), particularly when CSU is associated with angio-oedema and/or CIndU (Dermatology Life Quality Index > 10 in 30% of patients). Comorbidities, such as anxiety and depression, which are present in more than 30% of patients with CSU, compound HRQoL impairment. Severe pruritus and the unpredictable occurrence of weals and angio-oedema are responsible for sleep disorders; sexual dysfunction; limitations on daily life, work and sports activities; interfering with life within the family and in society; and patients' performance at school and work (6% absenteeism and 25% presenteeism). Apart from treatment costs, with annual values between 900 and 2400 purchasing power parity dollars (PPP$) in Europe and the USA, CU is associated with a high consumption of medical resources and other indirect costs, which may reach a total annual cost of PPP$ 15 550.

This update and revision of the international guideline for urticaria was developed following the methods recommended by Cochrane and the Grading of Recommendations Assessment, Development and Evaluation (GRADE) working group. It is a joint initiative of the Dermatology Section of the European Academy of Allergology and Clinical Immunology (EAACI), the Global Allergy and Asthma European Network (GALEN) and its Urticaria and Angioedema Centers of Reference and Excellence (UCAREs and ACAREs), the European Dermatology Forum (EDF; EuroGuiDerm), and the Asia Pacific Association of Allergy, Asthma and Clinical Immunology with the participation of 64 delegates of 50 national and international societies and from 31 countries. The consensus conference was held on 3 December 2020. This guideline was acknowledged and accepted by the European Union of Medical Specialists (UEMS). Urticaria is a frequent, mast-cell-driven disease that presents with wheals, angioedema, or both. The lifetime prevalence for acute urticaria is approximately 20%. Chronic urticaria, i.e. chronic spontaneous urticaria and chronic inducible urticaria, is disabling, impairs quality of life, and affects performance at work and school. This updated version of the international guideline for urticaria covers the definition and classification of urticaria and outlines expert-guided and evidence-based diagnostic and therapeutic approaches for the different subtypes of urticaria. This article is protected by copyright. All rights reserved.

Chronic spontaneous urticaria (CSU) is a debilitating mast cell-driven disease characterized by recurrent wheals and/or angioedema. Substantial progress has been made in dissecting the 2 main autoimmune mechanisms that drive the pathogenesis of CSU. Type I autoimmune (autoallergic) CSU is associated with IgE antibodies against autoantigens, for example, thyroid peroxidase and IL-24. Type IIb autoimmune CSU is mediated by autoantibodies that activate mast cells, for example, via IgE and FcεRI, and is present in less than 10% of patients with CSU when strict criteria are used, that is, triple positivity of autologous serum skin test, immunoassays for IgG autoantibodies, and basophil activation tests. A subpopulation of patients with CSU has both types. Type IIb autoimmune CSU is characterized by higher disease severity, concomitant autoimmune diseases, low levels of total IgE, elevated levels of IgG-anti-thyroid peroxidase, basopenia, eosinopenia, poor response to antihistamines and to omalizumab, and a good response to cyclosporine. Novel targeted therapies for CSU are under development such as ligelizumab, an anti-IgE, fenebrutinib and remibrutinib, Bruton's tyrosine kinase inhibitors, and dupilumab, an anti-IL-4Rα. Further studies should investigate the overlap between autoallergic and type IIb autoimmune CSU, optimize the diagnosis of both autoimmune endotypes using easy-to-perform, noninvasive, and inexpensive markers, and assess differences in response to therapy.

Chronic spontaneous urticaria (CSU) is defined as the spontaneous development of itchy hives and/or angioedema due to known or unknown causes that last for at least 6 weeks. At any given time, CSU is believed to affect 0.5–1% of the global population. Omalizumab (a recombinant, humanized anti-immunoglobulin-E antibody) is the only approved treatment for antihistamine refractory CSU. However, ~ 30% of patients remain symptomatic at licensed doses of omalizumab 150 mg and 300 mg, even after a treatment period of over 6 months. In the recent years, there have been several studies on updosing of the drug, suggesting that the individualized approach for urticaria treatment with omalizumab is useful. In this article, we provide an overview of these studies and the real-world data on omalizumab updosing as it became necessary to obtain complete CSU symptom control in a proportion of patients. Published observational studies (from June 2003 to October 2019) on the updosing of omalizumab in CSU were identified using PubMed and Ovid databases. Reports mainly show that updosing/dose adjustment evaluated with the assessment of disease activity (Urticaria Activity Score) and control (Urticaria Control Test) achieves better clinical response to omalizumab with a good safety profile in a pool of patients with CSU. These real-world data will provide an overview of updosing of omalizumab in CSU and aid in setting informed clinical practice treatment expectations., Novartis Pharma AG

Omalizumab is an effective and safe treatment for antihistamine resistant chronic spontaneous urticaria (CSU), however, long-term efficacy and safety remains unclear.To evaluate the efficacy and safety of omalizumab in CSU patients treated for long term and to identify long-term management strategies.We retrospectively analyzed demographic characteristics, clinical features, laboratory parameters and treatment outcomes of 41 CSU patients who received omalizumab for at least 3 years. Treatment responses were evaluated with urticaria control test (UCT). Treatment safety was evaluated by comparing laboratory findings before and three years after omalizumab initiation as well as considering patients' adverse event reports.The patients (mean age 40.46 years; 63.4% women) received omalizumab for an average of 41.93 months (mean 31.68 injections/patient). The mean baseline UCT score was 5.56 and average number of injections to reach UCT score ≥12 was 3.3. Nine patients (22%) responded insufficiently to 300 mg/4 weeks omalizumab and required updosing. Thirty-eight patients (92.7%) could tolerate longer dose intervals (4 weeks) and the dose interval was increased after a mean of 11.53 injections. There was no loss of efficacy of omalizumab. Sixteen patients (39%) had been retreated with omalizumab after a mean discontinuation time of 24 weeks. Five patients (12.2%) reported mild and transient adverse effects. Liver and renal function tests as well as full blood count before and after omalizumab were in normal ranges.For the long-term management of CSU, omalizumab is a safe and effective treatment which can be tailored according to patients' disease activity.

*Ateş, Can ( Aksaray, Yazar ), SciVal Topics Funding details Abstract Background: Chronic urticaria (CU) predominantly affects women, and sex hormones can modulate disease activity in female CU patients. As of now, the impact of pregnancy on CU is largely unknown. Aim: To analyze the course and features of CU during and after pregnancy. Patients and methods: PREG-CU is an international, multicenter study of the Urticaria Centers of Reference and Excellence (UCARE) network. Data were collected via a 47-item questionnaire completed by CU patients, who became pregnant within the last 3 years. Results: A total of 288 pregnancies of 288 CU patients from 13 countries were analyzed (mean age at pregnancy: 32.1 ± 6.1 years, duration of CU: 84.9 ± 74.5 months; CSU 66.9%, CSU + CIndU 20.3%, CIndU 12.8%).During pregnancy, 51.1% of patients rated their CU as improved, 28.9% as worse, and 20.0% as unchanged.CU exacerbations most commonly occurred exclusively during the third trimester (in 34 of 124 patients; 27.6%) or the first (28 of 124; 22.8%). The risk factors for worsening of CU during pregnancy were having mild disease and no angioedema before pregnancy, not taking treatment before pregnancy, CIndU, CU worsening during a previous pregnancy, treatment during pregnancy, and stress as a driver of exacerbations. After giving birth, urticaria disease activity remained unchanged in 43.8% of CU patients, whereas 37.4% and 18.1% experienced worsening and improvement, respectively. Conclusions: These results demonstrate the complex impact of pregnancy on the course of CU and help to better counsel patients who want to become pregnant and to manage CU during pregnancy.

The question how second-generation antihistamines (sgAHs) should be used when chronic spontaneous urticaria (CSU) is under control with omalizumab is still unanswered. This study aimed to investigate the effectiveness of as-needed sgAHs in patients with well-controlled urticaria under omalizumab treatment. Patients from four different urticaria centers who were treated with omalizumab 300 mg/4 weeks for at least 3 months, had well-controlled urticaria (Urticaria Control Test: 16 > UCT >= 12) and were using sgAHs only if needed, were included in this study. In order to assess effectiveness of sgAHs, change in the itch, hives, and total itch-hives scores before and after sgAHs were evaluated using modified urticaria activity score-twice daily. Fifty-three patients [38 female (71.7%)] with mean age 41.1 +/- 11.4 years were included in this study. Median sgAH intake per patient throughout the 4 week-intervals was 3 (2-5) tablets. sgAH intake decreased itch, hives and total itch-hives scores 45.7% +/- 52.9, 42.4% +/- 39.1, and 50.2% +/- 51.1, respectively (P < .001 for all). This decrease was similar in both isolated-urticaria and urticaria-and-angioedema phenotypes. Baseline IgE levels were positively correlated with the decrease of three symptom scores (r = 0.31, P = .05; r = 0.375, P = .017; r = 0.31, P = .05, respectively) that showed in patients with higher baseline total IgE levels, as needed sgAH intake decreased the symptom scores less. Our study showed that sgAHs may still be an effective option for the treatment of the intermittent symptoms in patients with well-controlled urticaria under omalizumab treatment. Baseline total IgE levels may be used as a potential biomarker for sgAH effectiveness in these patients.

This evidence- and consensus-based guideline was developed following the methods recommended by Cochrane and the Grading of Recommendations Assessment, Development and Evaluation (GRADE) working group. The conference was held on 1 December 2016. It is a joint initiative of the Dermatology Section of the European Academy of Allergology and Clinical Immunology (EAACI), the EU-founded network of excellence, the Global Allergy and Asthma European Network (GA²LEN), the European Dermatology Forum (EDF) and the World Allergy Organization (WAO) with the participation of 48 delegates of 42 national and international societies. This guideline was acknowledged and accepted by the European Union of Medical Specialists (UEMS). Urticaria is a frequent, mast cell-driven disease, presenting with wheals, angioedema, or both. The lifetime prevalence for acute urticaria is approximately 20%. Chronic spontaneous urticaria and other chronic forms of urticaria are disabling, impair quality of life and affect performance at work and school. This guideline covers the definition and classification of urticaria, taking into account the recent progress in identifying its causes, eliciting factors and pathomechanisms. In addition, it outlines evidence-based diagnostic and therapeutic approaches for the different subtypes of urticaria.

The recommended dose of omalizumab for the treatment of chronic spontaneous urticaria (CSU) is 300 mg every 4 weeks, but there is no recommendation for patients who do not benefit from this dose. Our aim is to present the experiences on the use of doses of omalizumab higher than those recommended for CSU patients and to propose a protocol for updosing. This was a retrospective analysis of patients treated with omalizumab for CSU from 2 urticaria centers in Istanbul and Barcelona. The weekly urticaria activity score and/or the Urticaria Control Test (UCT) were used to monitor response. In Barcelona, a stepwise updosing regimen was preferred (450 mg first, then increasing to 600 mg), while in Istanbul, direct updosing to 600 mg was chosen. In Istanbul, 81 (88%) patients were treated with 300 mg, while 11 (12%) received 600 mg of omalizumab. In Barcelona, 7 (8.8%), 45 (56.3%), 17 (21.3%), and 11 (13.8%) patients received 150, 300, 450, and 600 mg of omalizumab, respectively. Urticaria control was achieved in 82.6% of the patients with 300 mg and in 8.7% of the patients with 600 mg in Istanbul, while it was achieved with 150 mg in 10%, with 300 mg in 48.8%, with 450 mg in 16.3%, and with 600 mg in 6.3% of the patients in Barcelona. In total, 123 (71.5%) patients responded to 150–300 mg and 26 (15.1%) to 450–600 mg. When responders to 150–300 mg (n = 123) were compared with responders to 450–600 mg (n = 26), BMI was found to be higher, and pre-omalizumab UCT was found to be lower in patients receiving updosed omalizumab (p = 0.029). Baseline data of the patients, especially BMI and pre-oma UCT, might be useful to determine if the patient will require higher doses of omalizumab. We recommend a stepwise approach starting from 450 mg and then updosing to 600 mg in CSU patients who do not respond or partially respond to 300 mg of omalizumab after 3–6 months of treatment.

BACKGROUND: Autoimmune processes are considered to play a major role in the pathogenesis of chronic spontaneous urticaria (CSU). Very recently, interleukin 24 (IL-24) has been identified as an immunoglobulin E (IgE) autoantigen in CSU. Some studies revealed that notably autologous serum skin test (ASST)-positive CSU patients may benefit from autohemotherapy; however, the mechanisms of action remain unknown. We aimed to investigate the immunological effects of autologous serum injections in ASST-positive CSU patients.METHODS: Sixty-six ASST-positive CSU patients were treated with weekly intramuscular autologous serum injections for 8 weeks and followed up for 12 weeks. Urticaria activity score (UAS7) and Dermatology Life Quality Index (DLQI) were assessed. The ASST was done at baseline, week 9 and week 21. Serum samples (baseline, weeks 9, 13 and/or 21) were analysed for the levels of IgE-anti-IL-24 and immunoglobulin G (IgG)-anti-IL-24 via ELISA and their ability to release histamine in basophils [basophil histamine release assay (BHRA)].RESULTS: Autologous serum therapy resulted in a substantial improvement in disease activity and quality of life after 8 and 20 weeks. Twenty-eight percent and 34% of patients turned ASST-negative in weeks 9 and 21, respectively, but there was no link between their response to treatment and changes of ASST results. Also, no significant or relevant changes in BHRA were observed. In contrast, autologous serum therapy significantly decreased IgE-anti-IL-24 serum levels, but not IgG-anti-IL-24 serum levels, in responders but not in non-responders.CONCLUSIONS: Our findings suggest that the immunological effects of autologous serum therapy include a reduction in IgE-anti-IL24 autoantibodies, which may contribute to the pathogenesis of CSU.

BACKGROUND: GA²LEN, the Global Allergy and Asthma European Network, has recently launched a program for the development, interaction, and accreditation of centers of reference and excellence in special areas of allergy embedded in its overall quality management of allergy centers of excellence. The first area chosen is urticaria. Urticaria is a common and debilitating condition and can be a challenge for both patients and treating physicians, especially when chronic. Centers of reference and excellence in urticaria (UCAREs) can help to improve the management of hard-to-treat conditions such as urticaria.AIMS: Here, we describe the aims, the requirements and deliverables, the application process, and the audit and accreditation protocol for GA²LEN UCAREs.RESULTS: The main aims of GA²LEN UCAREs are to provide excellence in urticaria management, to increase the knowledge of urticaria by research and education, and to promote the awareness of urticaria by advocacy activities. To become a certified GA²LEN UCARE, urticaria centers have to apply and fulfill 32 requirements, defined by specific deliverables that are assessed during an audit visit.DISCUSSION AND CONCLUSION: The GA²LEN UCARE program will result in a strong network of urticaria specialists, promote urticaria research, and harmonize and improve urticaria management globally.

Symptomatic management of chronic spontaneous urticaria (CSU) basically depends on second-generation H1 antihistamines and omalizumab. Omalizumab is a game changer in the management, but still there is a need for new targets and new biologics targeting new pathways in the treatment which will provide long-lasting remission, which will be given orally and which will be cheaper. This review will focus on new biologics that are underway of production or are already under use for different disorders but could be beneficial for the treatment of Chronic urticaria.In this review, the treatment targets are classified according to the cells which are involved in the pathogenesis of CSU. Those are mast cells/basophils, B cells, T cells and eosinophils. The treatments that are under clinical trials for CSU are anti-IgE treatments such as ligelizumab, molecules targeting intracellular signaling pathways such as spleen tyrosine kinase inhibitors, surface inhibitory molecules such as siglec-8, anti-IL-1s such as canakinumab, Bruton kinase (BTK) inhibitors such as GDC-0853 and anti-IL-5s such as benralizumab and mepolizumab.The ongoing clinical trials on new targets of treatment hold new hopes not only for a better care of the disease but also a better understanding of the pathomechanisms lying underneath.

BACKGROUND/OBJECTIVES The effect of pediatric psoriasis on quality of life has been demonstrated, but data regarding its influence on caregiver quality of life are scarce. The objective was to investigate how psoriasis affects quality of life of children and their caregivers. METHODS This multicenter study included 129 children with psoriasis and their caregivers, who were family members accompanying patients to the clinic. Patient quality of life was measured using the Child Dermatology Life Quality Index. Caregiver quality of life was assessed using Dermatological Family Impact Scale, a 15-item questionnaire validated for use in the Turkish language. RESULTS Mean Child Dermatology Life Quality Index score was 7.6, indicating a moderate effect on patient quality of life. Symptoms and feelings were the most severely impaired domains of patient quality of life, and emotions was the most severely impaired domain of caregiver quality of life. Dermatological Family Impact Scale score was significantly correlated with Child Dermatology Life Quality Index (correlation coefficient [r] = .554, P

Background: Fric test is a useful tool for the diagnosis and threshold testing for symptomatic dermographism. When threshold testing is not available, Urticaria Control Test (UCT) and Dermatology Life Quality Index (DLQI) might be used to assess disease control and quality of life (QoL) impairment, respectively. Objectives: In this study, we aimed to describe a new scoring system for the Fric test and evaluate the correlations of Fric scores with UCT, DLQI, and other disease activity assessment scores. Method: Provocation test with Fric Test 4.0 was performed in all patients at referral and at the 4th week. We considered a 4-grade rating score for Fric Test (0–4) [Total Fric Score (TFS)]. A positive response with all of the four pins suggested severe dermographism (TFS = 4), while a wheal with only the largest pin (4.5 mm) was considered as milder disease (TFS = 1). Treatment responses were evaluated with Fric Test 4.0, UCT, patient’s global assessment of disease severity (PatGA-VAS), the physician’s global assessment of disease control (PhyGA-VAS), and DLQI at baseline and at the 4th week of treatment. The correlations of TFS with UCT, DLQI, PatGA-VAS, PhyGA-VAS at baseline as well as the changes in the mean scores after treatment (week 4) were performed. Results: The mean UCT and DLQI scores were 8.69 ± 3.40 and 7.88 ± 6.02 at the first visit. At the second visit, TFS decreased from a mean of 2.79 ± 1.68 to 1.91 ± 1.85 (p < 0.001), and UCT scores and PhyGA-VAS were increased (p < 0.001), while DLQI scores, PatGA-VAS, and pruritus scores decreased significantly (p = 0.002; p = 0.001; p = 0.012). There was a positive correlation between TFS and pruritus scores (r = 0.378) and DLQI scores (r = 0.392). TFS was found to have a negative correlation with UCT score (r = –0.283) and PhyGA-VAS (r = –0.347). Conclusions: This new Fric scoring system allows comparison with other tools and shows moderate correlations with most of the tools. Using disease-specific tools is recommended since they provide a subjective evaluation of disease severity, QoL impairment, and disease control.

Background In-transit metastases are dermal and subcutanous metastatic foci located between the tumor and the closest regional lymph node. Although in-transit metastasis has been commonly described for malignant melanoma, there have been some reports of in-transit metastases arising from primary cutaneous malignancies. The risk of development of in-transit metastases is higher in patients with high-risk squamous cell carcinoma. Case Report We present a case of in-transit metastasis in a nonimmunosuppressed patient with a primary cutaneous squamous cell carcinoma.

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.

Background: Chronic exposure to inorganic arsenic is an important public health problem and leads to hyperpigmentation, hyperkeratosis, Bowen’s disease and non-melanoma skin cancers in humans. Arsenic keratosis is the most common skin change that is associated with arsenic exposure. It may be an indicator of arsenic induced health hazards. Objective: To determine the needs of patients with arsenic keratosis by evaluating the impact of disease on the quality of life with respect to treatment and provide rehabilitation services. Methods: A total of 47 subjects with arsenic hyperkeratosis, were enrolled in this crossover study. The demographics, smoking status and the presence of chronic diseases diagnosed by the physician were recorded. Weight and height of the subjects were measured and BMI was calculated. The patients were asked to respond the visual analogue scale and EuroQoL-5D scales for assessing the pain and quality of life impairment associated with arsenic hyperkeratosis. Results: In the study group, moderate to severe problems were reported in 78.7% of patients for pain/uncomfortable condition domains and in 91.5% in anxiety/depression domains. Lower quality of life was reported in those with associated chronic disorders, at an advanced age, in thin patients and in those with severe lesions. Conclusion: According to our knowledge, our study is the first one measuring the quality of life in patients with arsenic keratosis in the literature. By this study, we tried to underline the guidance of health services to be developed towards the needs of patients and the need for the development of new health policies by determining the requirements of these patients. © 2017 Informa UK Limited, trading as Taylor & Francis Group.

Acromegaly is a systemic syndrome caused by overproduction of growth hormone. Cutaneous, endocrine, cardiovascular, skeletal, and respiratory systems are affected. Cutaneous changes in acromegaly relate to overexpression of growth hormone and insulin-like growth factor 1 on skin cells and adnexa. Dermal glycosaminoglycan accumulation and edema cause skin distention that is most prominent in the face, hands, and feet. Oily skin with large pores, hypertrichosis, and excessive sweating are common features. Pigmented skin tags, acanthosis nigricans, and psoriasis are also encountered. Cutaneous manifestations of acromegaly are various and prominent, and are an important clue for the early diagnosis and treatment of this high-morbidity disorder.

Background: Mucocutaneous manifestations of inflammatory bowel diseases are relatively common; the mean incidence is around 10% at the time of diagnosis. However, during follow-up, an increased variety of skin lesions, granulomatous cutaneous disease, reactive skin eruptions, nutritional defiencies, and other associated conditions may develop. Objective: The objective of this study was to evaluate the prevalence of the mucocutaneous manifestations and their association with gender, duration of disease, arthritis, location of the bowel disease, and disease activity. Methods: Fifty-six patients with ulcerative colitis (UC) and 36 patients with Crohn disease (CD) who were in follow-up in the Istanbul Medeniyet University Göztepe Training and Research Hospital Department of Gastroenterology were included in the study. Whole-body dermatologic examinations were performed for all patients, and patient files were evaluated for mucocutaneous manifestations. Results: Of the 92 patients, 49 (53.26%) presented with at least one mucocutaneous manifestation (58.9% of patients with UC and 44.4% of patients with CD). Of these, 38 (41.3%) had at least one reactive skin eruption. Aphthous stomatitis was noted in 33 patients (35.86%) and became the most common mucocutaneous manifestation. Granulomatous cutaneous diseases were detected in 18 patients (19.57%), and none of the patients had a nutritional deficiency–associated skin condition. Only 3 patients (3.26%) had erythema nodosum and 2 patients (2.17%) had pyoderma gangrenosum. Conclusions: We found that mucocutaneous manifestations of inflammatory bowel diseases are more common than thought and are more common in UC than in CD. No association was detected between mucocutaneous manifestions and gender, duration of disease, arthritis, location of the bowel disease, and activity of the disease.

Summary Brooke–Spiegler syndrome (BSS) is a rare, autosomal dominant disorder characterized by multiple adnexal tumours, especially trichoepitheliomas, cylindromas and occasionally spiradenomas. These lesions usually begin to appear in the second or third decade of life. Malignant transformation of pre-existing tumours may occur. In vivo reflectance-mode confocal microscopy (RCM) is a noninvasive method that can be used to visualize the epidermis and the upper dermis at almost histological resolution. It has been used to evaluate several skin conditions, especially malignant lesions, and has been reported to be useful for differentiating between malignant and benign skin tumours. Only a few studies have reported on the use of confocal microscopy to characterize the features of benign adnexal neoplasms. We present a patient with BSS who presented to our clinic with multiple skin tumours. The possible utility of RCM for identifying adnexal neoplasms is emphasized in this report, which also describes the observed microscopic features.

Background The recommended treatment approach in chronic inducible urticarias (CIndU) is the same as that for chronic spontaneous urticaria (CSU). But there is a lack of controlled trials assessing efficacy of available treatment options. Objective We aimed to evaluate the efficacy of treatment algorithm recommended by the guidelines and comparison of treatment responses in CIndU vs CSU. Methods This prospective parallel group controlled study included 70 CIndU and 66 CSU patients. The same treatment algorithm recommended by the European Academy of Allergology and Clinical Immunology/Global Allergy and Asthma European Network/European Dermatology Forum/World Allergy Organization (EAACI/GA 2 LEN/EDF/WAO) was implemented to both CSU and CIndU patients. Treatment responses of the groups were evaluated with urticaria control test (UCT) and dermatology life quality questionnaire (DLQI) scores at the 0, 4, 8, 12 and 24th weeks for CIndU and 0, 4, 12 and 24 weeks for CSU. Results Fourteen patients (20,9%) with CIndU and 25 (37,9%) with CSU responded to standard doses of H1-AHs which was significantly higher in the CSU group (p = 0,031, p 0,05). Twenty-seven (40,3%) of CIndU patients and 21 (31,8%) of CSU patients were diagnosed as AH-resistant urticaria. Omalizumab was administered to 15 CSU patients and 17 CIndU patients. Response rates to omalizumab were similar in both groups. Total response rates increased from 37,9% (n:25) to 68,2% (n:45) with the 2nd line treatments in CSU group while it increased from 20,9% (n:14) to 59,7% (n:40) in CIndU group. When omalizumab was introduced to AH-refractory cases as a 3rd line treatment, total response rates evaluated at the 12th week were 76,1% (n:51) in patients with CIndU and 83,3% (n:55) in CSU. Continuing omalizumab treatment for 24 weeks increased response rates in patients who were unresponsive at week 12. Conclusion CIndU seem to be more resistant to standard doses of AHs and higher doses of AHs are required for the control of symptoms. The same guidelines for CSU may be implemented to patients with CIndU.

Recent demonstration of circulating anti-IgG antibodies towards IgE and its receptor (FcϵRI) has led to an interest in inducing tolerance to circulating histamine-releasing factors with autologous blood injections as a treatment option in chronic spontaneous urticaria (CU). The aim of the study was to assess the efficacy of autologous whole blood (AWB) and autologous serum (AS) injections in patients with CU compared to placebo.A total of 88 CU patients with (+) autologous serum skin test (ASST) (59) and (-) ASST (29) were randomized into three parallel subgroups and were treated with weekly injections of AWB, AS or placebo for 10 weeks. Clinical assessments included urticaria activity score (UAS) and dermatology life quality index.In ASST (+) patients, the percentages of patients with30% improvement in UAS and DLQI were 85% and 90% in AWB group, 65% and 65% in AS group and 79% and 90% in placebo group, respectively. In ASST (-) patients, these figures were 67% and 89% in the AWB group, 80% and 80% in the AS group and 60% and 70% in the placebo group. The intergroup difference for complete subsidence was not statistically significant.Even though we could not show a better efficacy than placebo, autohemotherapy resulted in a marked decrease in disease activity and improvement in quality of life scores in CU patients.

Recent concerns have arisen about the specificity and interpretation of the autologous serum skin test (ASST), suggesting that ASST might produce false-positive results, and proposing the use of autologous plasma (APST) instead for intradermal testing in autoreactive urticaria. We investigated autoreactivity to autologous plasma and compared the results for reproducibility, sensitivity, specificity and accuracy and evaluated their association with quality of life and anti-TPO antibodies. 70 adults with chronic spontaneous urticaria (CU) and 62 controls underwent testing with ASST and APST and the tests were repeated two days after the first visit. Blood tests measured anti-TPO levels. Disease activity was assessed by urticaria activity score (UAS-7) and quality of life impairment was assessed by DLQI and CU-Q(2)oL. There were no statistically significant differences between ASST (+) and ASST (-) and also APST (+) and APST (-) patients with regard to disease duration, anti-TPO antibodies, urticaria activity scores, DLQI scores and CU-Q(2)oL scores. The results of first ASST and APST were well correlated with the results of second ASST and APST. The specificity of the two tests was similar, while ASST had a higher sensitivity and accuracy. Our results showed that there is no need to use autologous plasma instead of autologous serum for intradermal testing in CU.

Vitiligo is an acquired, pigmentary skin disorder that affects about 0.1-4.0% of the population. In this study, we aimed to investigate the disease features such as age of onset, disease duration, clinical and sociodemographic characteristics, and laboratory parameters of patients with vitiligo. A hundred patients who were in follow-up for vitiligo between the period of June 2013 and May 2014 were included in the study. The clinical features and laboratory parameters were retrospectively obtained from the records of the patients. The mean age was 34.9±16.8 years. The most common clinical types were focal and acrofacial. Facial involvement was the most common localization. Forty-five (45%) patients had an associated systemic disease. Autoimmune thyroid disease, essential hypertension, and alopecia areata, which were observed in 28%, 8%, and 5% of patients, respectively, were the most common associated diseases. Twenty-one percent of the patients had low ferritin levels, 20% had low iron levels, 12% had low vitamin B12 levels, and 1% had low folic acid levels. The prevalence of anti-TG (anti-thyroglobulin) and anti-TPO (anti-thyroid peroxidase) antibodies were found 17% and 27% of the patients, respectively. We found that the clinical characteristics of vitiligo in our patients were similar to those in other studies. We observed laboratory abnormalities and accompanying diseases associated with vitiligo. Therefore we conclude that laboratory examinations including thyroid antibodies and regular follow-up of these patients are essential.

Vitamin D plays a key role in the immune responses generated by lymphocytes and antigen-presenting cells. Decreased vitamin 25-hydroxyvitamin D (25(OH)D) levels have been implicated in several allergic disorders and association between 25(OH)D levels and chronic urticaria (CU) symptom scores has been evaluated in a few studies. This study was performed to assess the effects of vitamin D supplementation on the symptoms and quality of life scores in chronic spontaneous urticaria (CSU) and to vitamin D levels in CSU patients in comparison with controls.Fifty-eight CSU patients and forty-five controls were included in the study. The patients were divided into two groups according to severity of the disease; as mild/moderate and severe urticaria. Serum 25-hydroxyvitamin D (25(OH)D) concentrations were measured in serum of CSU patients and compared with the control groups. In patients with 25(OH)D concentrations lower than 30 µg/L, 300 000 IU/month of vitamin D3 supplementation was added to standard therapy. The clinical improvement was evaluated after 3 months with urticaria activity score (UAS4) and Chronic Urticaria Quality of Life Questionnaire (CU-Q2oL).Serum 25(OH)D concentration was significantly lower in CSU group compared to healthy subjects (p 0.001). The prevalence of vitamin D deficiency (20 (µg/L) and insufficiency (30 µg/L) was significantly higher in CSU patients than control groups. In addition, 25(OH)D concentrations were significantly lower in both mild-moderate and severe CSU patients than those of the controls (p = 0.011 and p 0.001, respectively). Ninety eight percent of patients (25(OH)D 30 µg/L) were treated with vitamin D3 (300 000 IU/month) supplementation, and after 12 weeks, these patients showed significant improvements in UAS4 and CU-Q2oL scores.This study support the contributing and beneficial effects of vitamin D in the treatment of CU. Replacement of vitamin D may provide improvement in both the severity of symptoms and the quality of life scores in these patients.

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.

1.Department of Pediatrics, Division of Neonatology, Goztepe Education and Research Hospital, Istanbul Medeniyet University,Istanbul, Turkey2.Department of Pediatrics, Goztepe Education and Research Hospital, Istanbul Medeniyet University, Istanbul, Turkey3.Department of Dermatology, Goztepe Education and Research Hospital, Istanbul Medeniyet University, Istanbul, Turkey4.Department of Pathology, Goztepe Education and Research Hospital, Istanbul Medeniyet University, Istanbul, TurkeyCorrespondenceIlke Mungan Akin, Barbaros Mah. Bilgen Konutlari B-BlokD:36, Kosuyolu, Uskudar, Istanbul 34662, Turkey.Tel: +90 532 707 21 50 |Fax: + 90 216 332 28 20 |Email: ilkemungan@gmail.comReceived9 July 2012; revised 5 December 2012;accepted 14 December 2012.DOI:10.1111/apa.12137

Background. Psoriasis is a chronic inflammatory skin disorder in which proinflammatory cytokines including IL-6 and TNF-αincrease 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.

Chronic spontaneous urticaria has a substantial impact on patients' quality of life. The first disease-specific tool to assess quality of life impairment in this condition, the Chronic Urticaria Quality of Life Questionnaire (CU-Q2oL), was developed recently. The aim of this study was to adapt the original Italian version to the Turkish language and to evaluate its reliability, validity, and sensitivity to change. The Turkish version was developed by performing forward- and back-translation. It was then applied to 140 consecutive patients with chronic spontaneous urticaria, along with the Dermatology Life Quality Index and the Skindex-29. Disease activity was assessed using the Urticaria Activity Score. Sensitivity to change was measured in 101 patients, who completed the instruments twice at intervals of 4 weeks. Confirmatory factor analysis demonstrated that the six-scale structure of the original Italian version ("pruritus", "swelling", "impact on life activities", "sleep problems", "limits", "looks") can be retained in the Turkish instrument. Analysis regarding convergent validity showed good correlations of the Turkish CU-Q2oL with the other instruments. In addition, it was found to discriminate well between patients with different levels of urticaria activity, and to be sensitive to change. In conclusion, the Turkish version of CU-Q2oL is a reliable, valid, and sensitive instrument, which will help to characterize better the clinical impact of chronic spontaneous urticaria and treatment outcomes in Turkish patients. Its identical scale structure to that of other CU-Q2oL instruments makes it ideal for cross-cultural comparisons and for its application in future national and multinational studies.

Patients with Behçet's disease (BD) have been recognized to be at an increased risk of thrombosis and thrombotic complications have been reported in 12%-40% of patients. The precise pathogenetic mechanisms underlying the thrombotic tendency of BD are not known. In recent researches, it is reported that procoagulant mutations might play a role in thrombotic process in BD patients. We aimed to evaluate the frequency of activated protein C resistance (APCR) in our BD patients and to investigate the association between thrombophlebitis and APCR. The study included 116 patients with BD who fulfilled the International Study Group criteria and 70 healthy individuals as a control group. APCR levels were measured by the clotting method. APCR levels were 129.63 ± 39.70 and 152.26 ± 22.62 in BD patients and control group, respectively (P0.01). APCR was found in 47.4% and 8.6% of BD patients and control group, respectively (P0.01). There was no statistically significant difference regarding APCR levels between patients with thrombophlebitis and without thrombophlebitis (46.4% vs. 48.3%). We found the frequency of APCR to be increased in BD patients with or without thrombophlebitis. The lack of association between thrombophlebitis and APCR in our series of BD patients suggests that some factors like endothelial abnormalities other than thrombophilia play a major role in the pathogenesis of thrombosis in BD.

Background: Strong negative influence upon the quality of life in chronic urticaria is well proved. Before the GA 2 LEN Chronic Urticaria Quality of Life Questionnaire (CU-Q2oL) was introduced, the quality of life in chronic urticaria had been measured with general or dermatology specific questionnaires. CU-Q2oL was initially developed in Italy and consisted of 23 items divided into 6 quality of life dimensions. Objective: The aim of our study was to adapt the Polish version of CU-Q2oL and to provide initial results from the Polish sample. Methods: To prepare the Polish version forward and back translation was prepared. After cognitive debriefing, we collected a group of 126 chronic urticaria patients who completed Polish CU-Q2oL, Dermatology Life Quality Index (DLQI) and Skindex-29 questionnaire. Disease severity was assessed with Urticaria Activity Score (UAS). We performed the factorial analysis to identify CU-Q2oL subscales in our study, internal consistency and convergent validity assessment as well as factors driving the results. Moreover, we analysed tool’s reproducibility and responsiveness. Results: The factor analysis resulted in six subscales of Polish CU-Q2oL version with satisfying face validity: Itching, Swelling/Mental status, Functioning, Sleep, Eating/Limits, Embarrassment. All subscales presented recommended internal consistency and convergent validity. Disease severity was the only factor predicting results of all the subscales. Polish CU-Q2oL version was reproducible and sensitive to change. We noticed the highest quality of life impairment in Itching and Embarrassment subscales whereas Eating/Limits was the least affected. Conclusions: Our study supports reliability, responsiveness and validity of the Polish version of CU-Q2oL – easy in use, non time-consuming instrument to be used in research, clinical management and treatment outcome assessment and is one more step to confirm quality of life impairment in chronic urticaria.

Verrucous hemangioma is an uncommon capillary or cavernous hemangioma with reactive epidermal changes of hyperkeratosis, acanthosis, and papillomatosis. These growths are usually located on the lower extremities and involvement is generally unilateral. A diagnosis of verrucous hemangioma should be considered in lesions especially with a hyperkeratotic nodular appearance located on the lower extremities.

Warty dyskeratoma is usually characterized by solitary papules or nodules which reveal acantholytic dyskeratosis histopathologically. Warty dyskeratoma most commonly presents as a single lesion, but there are a few case reports of patients with multiple lesions. Herein we report a female patient presenting with multiple verrucous papules on the scalp diagnosed clinically and histopathologically as warty dyskeratoma.

Summary Pigmented purpuric dermatoses are a group of dermatoses characterized clinically by pinpoint petechia and purpura on a brown, red or yellow base. Five diseases are included in the group of pigmented purpuric eruptions which significantly overlap clinically and histologically. Phototherapy in various forms of purpuric dermatoses has been reported in literature previously. Here we present a case of pigmented purpuric lichenoid dermatitis which rapidly cleared with narrowband UVB therapy.

A 20-year-old woman presented with a 1.5-year history of pruritic papules on her vulva. There was no past history of eczema or contact sensitization. Cutaneous examination revealed multiple, 1-3 mm sized, skin-colored papules, distributed bilaterally over the labia majora (Fig. i). No other similar lesions were detected on the rest of the body. Histological examination of the lesion showed a tumoral lesion containing ductal structures formed by epithelial cells some of which were clear cytoplasm embedded in fibrotic stroma (Fig. 2). This appearance was diagnostic of a syringoma.

Reticulate Acropigmentation of Kitamura (RAPK) was first described in Japan and most cases reported have been in patients of Asian ethnic groups. Angulated, slightly atrophic, hyperpigmented macules that are arranged in a reticulate pattern are typically found on the dorsal hands and feet. The condition is inherited in an autosomal dominant fashion and skin changes begin to develop during childhood. We report RAPK in a mother and daughter who are from a non-Asian ethnic group.

Livedoid vasculopathy is characterized by painful purpuric lesions on the extremities which frequently ulcerate and heal with atrophic scarring. It has been considered to be a vasculitic process but now there has been increasing evidence that abnormal coagulation plays a major role in the pathogenesis of this rare disorder. We report the case of a 19-year-old male who had been suffering from recurrent painful ulcerations on both lower legs with atrophic scars for 4 years. A biopsy specimen revealed vasculopathy, and laboratory studies showed resistance to activated protein C with factor V Leiden mutation. Treatment with oral warfarin led to rapid improvement of the lesions.