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Chronic spontaneous urticaria (CSU) is a typical example of an intractable skin disease with no clear cause and significantly affects daily life of patients. Because CSU is a human-specific disease and lacks proper animal model, there are many questions regarding its pathophysiological dynamics. On the other hand, most clinical symptoms of urticaria are notable as dynamic appearance of skin eruptions called wheals. In this study, we explored dynamics of wheal by dividing it into three phases using a mathematical model: onset, development, and disappearance. Our results suggest that CSU onset is critically associated with endovascular dynamics triggered by basophils positive feedback. In contrast, the development phase is regulated by mast cell dynamics via vascular gap formation. We also suggest a disappearance mechanism of skin eruptions in CSU through an extension of the mathematical model using qualitative and quantitative comparisons of wheal expansion data of real patients with urticaria. Our results suggest that the wheal dynamics of the three phases and CSU development are hierarchically related to endovascular and extravascular pathophysiological networks., Competing Interests: Declarations Code availability Numerical code is made available online on GitHub (https://github.com/seirin-lee/urticaria-code) and all other data are available from the corresponding author (SSL) on reasonable request. Conflict of interest The authors declare no Conflict of interest., (© 2024. The Author(s).)

Objectives: To describe the emergency department (ED) triage of anaphylaxis patients based on the Emergency Severity Index (ESI), assess the association between ESI triage level and ED epinephrine administration, and determine characteristics associated with lower acuity triage ESI assignment (levels 3 and 4)., Methods: We conducted a cohort study of adult and pediatric anaphylaxis patients between September 2010 and September 2018 at an academic ED. Patient characteristics and management were compared between Emergency Severity Index (ESI) triage level 1 or 2 versus levels 3 or 4 using logistic regression analysis. We adhered to STROBE reporting guidelines., Results: A total of 1090 patient visits were included. There were 26 (2%), 515 (47%), 489 (45%), and 60 (6%) visits that were assigned an ESI triage level of 1, 2, 3, and 4, respectively. Epinephrine was administered in the ED to 53% of patients triaged ESI level 1 or 2 and to 40% of patients triaged ESI level 3 or 4. Patients who were assigned a lower acuity ESI level of 3 or 4 had a longer median time from ED arrival to epinephrine administration compared to those with a higher acuity ESI level of 1 or 2 (28 min compared to 13 min, p < .001). A lower acuity ESI level was more likely to be assigned to visits with a chief concern of hives, rash, or pruritus (OR 2.33 [95% CI, 1.20-4.53]) and less likely to be assigned to visits among adults (OR, 0.43 [0.31-0.60]), patients who received epinephrine from emergency medical services (OR 0.56 [0.38-0.82]), presented with posterior pharyngeal or uvular angioedema (OR, 0.56 [0.38-0.82]), hypoxemia (OR, 0.34 [0.18-0.64]), or increased heart (OR 0.83 [0.73-0.95]) or respiratory (OR 0.70 [0.60-0.82]) rates., Conclusion: Patients triaged to lower acuity ESI levels experienced delays in ED epinephrine administration. Adult and pediatric patients with skin-related chief concerns were more likely to be to be assigned lower acuity ESI levels. Further studies are needed to identify interventions that will improve ED anaphylaxis triage., Competing Interests: Declaration of Competing Interest R.L. Campbell has been a consultant for Bryn Pharma. The rest of the authors declare that they have no relevant conflicts of interest., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Angioedema without wheals (urticaria) represents a heterogeneous group of clinically indistinguishable diseases of hereditary or acquired etiology. Hereditary angioedema is a rare inherited condition leading to recurrent, sometimes life-threatening angioedema attacks in subcutaneous tissues and gastrointestinal and oropharyngeal mucosa dating back to childhood or adolescence. Most of these patients have mutations in the SERPING1 gene, causing either low C1 inhibitor production (hereditary angioedema with C1 inhibitor deficiency type I) or the production of dysfunctional C1 inhibitor (hereditary angioedema with C1 inhibitor deficiency type II). Hereditary angioedema with normal C1 inhibitor has been defined later. Although C1 inhibitor concentration and function are in the normal range, it leads to typical hereditary angioedema symptoms owing to mutations in FXII, PLG, ANGPT1, KNG1, and MYOF genes. Patients who exhibit none of these genetic mutations despite having a similar clinical presentation are classified as having unknown hereditary angioedema. Fewer than 1 in 10 patients with C1 inhibitor deficiency have acquired angioedema with C1 inhibitor deficiency. The clinical presentation is very similar to that of hereditary angioedema, making it difficult to distinguish these 2 conditions clinically. Unlike hereditary angioedema, there are no genetic mutations, and family history and symptoms tend to appear later in life. Acquired angioedema with C1 inhibitor deficiency is commonly associated with lymphoproliferative and autoimmune diseases. Angioedema attacks might start 1 year before the underlying disease in acquired angioedema with C1 inhibitor deficiency. Approximately half of the patients admitted to the hospital for acute angioedema are patients receiving angiotensin-converting enzyme (ACE) inhibitor therapy. Angioedema typically occurs on the lips, tongue, mouth, pharynx, and subglottic regions. Patients may require hospitalization and intensive care monitoring owing to airway involvement. Idiopathic histaminergic acquired angioedema may be diagnosed only when any possible causes of histaminergic angioedema are excluded (foods, drugs, animal dander, aeroallergens, insect stings, latex, and others), and the symptoms respond well to antihistamine treatment. Idiopathic nonhistaminergic acquired angioedema should be considered when all other types of recurrent angioedema have been ruled out and patients do not respond to high-dose antihistamines. The lack of a standard biochemical laboratory test for patients with idiopathic histaminergic acquired angioedema, idiopathic nonhistaminergic acquired angioedema, angiotensin-converting enzyme inhibitor-induced acquired angioedema, and hereditary angioedema with normal C1 inhibitor makes the diagnosis more challenging. Future efforts should focus on increasing awareness of all the rare types of angioedema among physicians and developing more straightforward and more accessible diagnostic methods.

Patients with coronavirus disease 2019 (COVID-19) present with multisystem signs and symptoms, including dermatologic manifestations. The recent literature has revealed that dermatologic manifestations of COVID-19 often are early onset and provide helpful cues to a timely diagnosis. We compiled the relevant emerging literature regarding the dermatologic manifestations of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) so that physicians can be aware of the various clinical cutaneous presentations in this time of high incidence of COVID-19.

The severe acute respiratory syndrome coronavirus two (SARS-CoV-2), which causes the 2019 coronavirus disease (COVID-19), has infected patients worldwide. Physicians have increasingly identified cutaneous findings as a significant clinical manifestation of COVID-19. In this review, we describe the clinical presentation, onset, duration, associated symptoms, treatment, and outcome of cutaneous manifestations thus far reported to be related to COVID-19. We have included data from 63 studies and subdivided reported cutaneous manifestations into the categories of viral exanthem, urticarial, vesicular, chilblains/chilblains-like, non-chilblains vasculopathy-related, pityriasis rosea-like, erythema multiforme-like, Kawasaki/Kawasaki-like disease, and others. Physicians should be aware of the known common cutaneous manifestations of COVID-19 and future research is required to better understand the pathophysiology and prognosis of each COVID-19-related skin manifestation.

This is a case of a patient who presented with an urticarial rash 48 hours before developing symptoms of fever and a continuous cough. She subsequently developed angioedema of her lips and hands before testing positive for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. Urticarial rashes occurring 48 hours before other symptoms of COVID-19 infection have been documented. This case demonstrates the importance of heightened awareness that not all urticarial rashes represent spontaneous urticaria and as a consequence, this may result in misdiagnosis and ultimately delayed diagnosis. This is the first reported case in the literature of urticaria with angioedema as a prodromal phenomenon of COVID-19., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2020. No commercial re-use. See rights and permissions. Published by BMJ.)

Osteoporosis (OP) is defined as a generalized skeletal disease characterized by low bone mass and an alteration of the microarchitecture that lead to an increase in bone fragility and, therefore, an increased risk of fractures. It must be considered today as a true public health problem and the most widespread metabolic bone disease that affects more than 200 million people worldwide. Under physiological conditions, there is a balance between bone formation and bone resorption necessary for skeletal homeostasis. In pathological situations, this balance is altered in favor of osteoclast (OC)-mediated bone resorption. During chronic inflammation, the balance between bone formation and bone resorption may be considerably affected, contributing to a net prevalence of osteoclastogenesis. Skin diseases are the fourth cause of human disease in the world, affecting approximately one third of the world's population with a prevalence in elderly men. Inflammation and the various associated cytokine patterns are the basis of both osteoporosis and most skin pathologies. Moreover, dermatological patients also undergo local or systemic treatments with glucocorticoids and immunosuppressants that could increase the risk of osteoporosis. Therefore, particular attention should be paid to bone health in these patients. The purpose of the present review is to take stock of the knowledge in this still quite unexplored field, despite the frequency of such conditions in clinical practice.

: Wine, beer, liquor, and spirits are widely consumed in many cultures across the globe, and for some individuals, ingestion, cutaneous contact, or other exposure can lead to dermatologic findings. However, there currently exist no comprehensive reviews on alcohol-related dermatitis. Herein, we will provide an overview of alcohol-related dermatitis and contact urticaria, including the epidemiology and clinical manifestations, potential allergens found in alcoholic beverages, testing approaches, and strategies for allergen avoidance.

Urticaria is a common skin disorder characterized by the rapid appearance and disappearance of local skin edema and flares with itching. It is characterized by various macroscopic skin eruptions unique to patients and/or subtypes of urticaria with respect to shape, size, color, and/or duration of eruptions. Nevertheless, the mechanism underlying multifarious eruptions in urticaria is largely unknown. The eruptions are believed to be evoked by histamine release from mast cells in the skin. However, the majority of visible characteristics of urticaria cannot be explained by a simple injection of histamine to the skin. To explain the multifarious eruptions of urticaria, we developed a single reaction-diffusion model suggesting the self-activation and self-inhibition regulation of histamine release from mast cells. Using the model, we found that various geometrical shapes of eruptions typically observed in patients can be explained by the model parameters and randomness or strength of the initial stimuli to mast cells. Furthermore, we verified that the wheal-expanding speed of urticaria, which is shown to be much smaller than that of the intradermal injection experimental system may be explained by our model and a simple diffusion equation. Our study suggests that the simple reaction-diffusion dynamics, including the independent self-activating and -inhibitory regulation of histamine release, may account for the essential mechanism underlying the formation of multifarious eruptions in urticaria., Competing Interests: The authors have declared that no competing interests exist.

Objective: Little is known regarding food anaphylaxis in infancy. We aimed to describe specificities of food anaphylaxis in infants (≤12 months) as compared to preschool children (1-6 years)., Methods: We conducted a retrospective study of all food anaphylaxis cases recorded by the Allergy Vigilance Network from 2002 to 2018, in preschool children focusing on infants., Results: Of 1951 food anaphylaxis reactions, 61 (3%) occurred in infants and 386 (20%) in preschool children. Two infants had two anaphylaxis reactions; thus, we analyzed data among 59 infants (male: 51%; mean age: 6 months [SD: 2.9]); 31% had a history of atopic dermatitis, 11% of previous food allergy. The main food allergens were cow's milk (59%), hen's egg (20%), wheat (7%) and peanut (3%) in infants as compared with peanut (27%) and cashew (23%) in preschool children. Anaphylaxis occurred in 28/61 (46%) cases at the first cow's milk intake after breastfeeding discontinuation. Clinical manifestations were mainly mucocutaneous (79%), gastrointestinal (49%), respiratory (48%) and cardiovascular (21%); 25% of infants received adrenaline. Hives, hypotension and neurologic symptoms were more likely to be reported in infants than in preschool children (P = .02; P = .004; P = .002, respectively). Antihistamines and corticosteroids were more often prescribed in preschool children than in infants (P = .005; P = .025, respectively)., Conclusion: Our study found that in infants presenting with their first food allergy, in a setting with a high rate of infant formula use, the most predominant trigger was cow's milk. As compared to older preschool children, hives, hypotonia and hypotension were more likely to be reported in infants. We believe that this represents a distinct food anaphylaxis phenotype that can further support developing the clinical anaphylaxis criteria in infants., (© 2019 John Wiley & Sons Ltd.)

Introduction : Food-dependent, exercise-induced anaphylaxis (FDEIA) is a rare type of anaphylaxis with a growing incidence. Although the precise mechanism by which the patient reacts only in a combination of a culprit food and cofactors are not currently understood, many advances in diagnosis and management have been made since their first description. Areas covered : A literature search in PubMed was performed to review the diagnosis and management of FDEIA. Clinicians should have a high level of suspicion for identification of the culprit foods and the cofactors involved. Component-resolved diagnosis and more accurate provocation tests have revolutionized the diagnosis accuracy. Management is not easy and involves educating the patient to evict the combination of exposure to the culprit foods and the cofactors that elicit anaphylaxis, and how to act and treat if a reaction occurs. Expert opinion : FDEIA is currently misdiagnosed and the authors believe that there are many FDEIA patients labelled as idiopathic anaphylaxis with unnecessary evictions and with a poor quality of life because of the fear of an imminent reaction. Due to recent advances in diagnostic tools and the use of monoclonal antibodies for prophylaxis in persistent cases, FDEIA can have a better prognosis improving the quality of life of the patients and their families.

Strongyloides stercoralis , a worldwide-distributed soil-transmitted helminth, causes chronic infection which may be life threatening. Limitations of diagnostic tests and nonspecificity of symptoms have hampered the estimation of the global morbidity due to strongyloidiasis. This work aimed at assessing S. stercoralis -associated morbidity through a systematic review and meta-analysis of the available literature. MEDLINE, Embase, CENTRAL, LILACS, and trial registries (WHO portal) were searched. The study quality was assessed using the Newcastle-Ottawa scale. Odds ratios (ORs) of the association between symptoms and infection status and frequency of infection-associated symptoms were calculated. Six articles from five countries, including 6,014 individuals, were included in the meta-analysis-three were of low quality, one of high quality, and two of very high quality. Abdominal pain (OR 1.74 [CI 1.07-2.94]), diarrhea (OR 1.66 [CI 1.09-2.55]), and urticaria (OR 1.73 [CI 1.22-2.44]) were associated with infection. In 17 eligible studies, these symptoms were reported by a large proportion of the individuals with strongyloidiasis-abdominal pain by 53.1% individuals, diarrhea by 41.6%, and urticaria by 27.8%. After removing the low-quality studies, urticaria remained the only symptom significantly associated with S. stercoralis infection (OR 1.42 [CI 1.24-1.61]). Limitations of evidence included the low number and quality of studies. Our findings especially highlight the appalling knowledge gap about clinical manifestations of this common yet neglected soil-transmitted helminthiasis. Further studies focusing on morbidity and risk factors for dissemination and mortality due to strongyloidiasis are absolutely needed to quantify the burden of S. stercoralis infection and inform public health policies.

Background: Chronic spontaneous urticaria (CSU) is defined as spontaneous occurrence of wheals and/or angioedema for ≥6 weeks. Omalizumab is a monoclonal anti-IgE antibody effective in refractory CSU, but its mechanism of action and markers predictive of response remain not completely defined., Objectives: To correlate baseline levels of two proposed biomarkers, total IgE (bIgE) and d-dimer (bd-dimer), and clinical parameters to omalizumab response and to relapses after drug withdrawal., Methods: In this retrospective Italian multicentre study, clinical data were collected in 470 CSU patients, and bIgE and bd-dimer were measured in 340 and 342 patients, respectively. Disease activity was determined by Urticaria Activity Score 7 (UAS7) at week 1 and 12 after omalizumab starting. Relapses were evaluated during a 2- and 3-month interval after a first and a second course of treatment, respectively., Results: bIgE correlated to a good response to omalizumab since levels were significantly higher in responders than non-responders (P = 0.0002). Conversely, bd-dimer did not correlate to response. There was no correlation between both bIgE and d-dimer and either first or second relapse. Disease duration was significantly longer in patients who experienced either first or second relapse (P < 0.0001 and P = 0.0105, respectively), while baseline UAS7 correlated only to first relapse (P = 0.0023)., Conclusions: Our study confirms bIgE as a reliable biomarker predicting response to omalizumab in CSU, while it does not support the usefulness of bd-dimer unlike previous findings. CSU duration before omalizumab and baseline UAS7 may be clinical markers of relapse risk., (© 2018 European Academy of Dermatology and Venereology.)

Water-related dermatoses are a spectrum of diseases that are associated with water exposure. They result either from the direct influence of water or after injury from water inhabitants. In this review, clinical entities that manifest after water exposure (aquagenic pruritus, aquagenic urticaria, and aquagenic wrinkling of the palms) will be discussed with particular focus on the reported pathogenesis. Aquatic organisms and activities can be linked to cutaneous injuries that require identification and distinct management. Marine and freshwater dermatoses were summarized to provide physicians with easy access to the causative organism, method of injury, and appropriate management., (© 2018 The International Society of Dermatology.)

Background: Quality of life, which is impaired in patients with chronic spontaneous urticaria (CSU), is influenced by comorbid mental disorders. Headaches could be another comorbid mental disorder that affects quality of life in children with CSU., Objectives: To investigate the effect of headaches on urticaria symptoms, disease activity and quality of life in children with CSU., Methods: A total of 83 patients with CSU were enrolled in the study and were separated into two groups as those with or without headache. Demographic and clinical characteristics were studied with the Urticaria Activity Score (UAS7), Urticaria Control test (UCT) and Chronic Urticaria Quality of Life Questionnaire (CU-Q2QoL). The headache questionnaire designed according to the Department of International Classification of Headache Disorders, second edition (ICHD-II) was used and VAS (Visual Analogue Scale) and NRS (Numerical Rating Scale) were used to assess the pain measurement. In patients diagnosed with migraine, the paediatric Migraine Disability Assessment Scale (PedMIDAS) was applied., Results: CU-QoL total scores were significantly higher in patients with CSU with headache than in those without headache (p=0.015). In the five domains of CU-QoL, impact of daily life activities domain and sleep problems domain had higher scores in CSU with headache (p=0.008, 0.028, respectively). There was no significant relationship between UCT, UAS and CU-QoL and headache severity (p<0.05). No differences were found between the groups in respect of duration of urticaria, UAS7 and UCT., Conclusion: Headache may be an important factor that affects and impairs quality of life in children with chronic urticaria., (Copyright © 2018 SEICAP. Published by Elsevier España, S.L.U. All rights reserved.)

Background: The rate of true vaccine allergy is unknown. Children with potential IgE-mediated adverse events following immunization (AEFI) should undergo allergy investigation that may include skin testing or challenge. Previous protocols tend to be highly conservative and often suggest invasive testing for all, a practice not evidence based, technically difficult, and unpleasant in children. It has more recently been suggested that skin testing may be restricted to those with allergic-like events within the first hour and those with a history of anaphylaxis., Objective: We aimed to describe the outcome of vaccine skin testing and challenge in children referred to a tertiary pediatric hospital with a potential IgE-mediated AEFI. The secondary aim was to identify any significant risk factors for vaccine allergy., Methods: A retrospective review of all children (<18 years) who underwent vaccine skin testing (skin prick testing or intradermal testing [IDT]) or challenge over a 5-year period (May 1, 2011, to April 30, 2016) at the Royal Children's Hospital Melbourne is presented., Results: There were 95 admissions in 73 children. Eight percent (6 of 73) of children had confirmed vaccine allergy (positive skin testing or challenge to the index vaccination). Two had positive IDT to a suspect vaccine but challenge negative to an alternative brand vaccine. Two had negative IDT but subsequent positive challenge and two had immediate urticaria on challenge without prior skin testing. All children in the positive group either had index reaction within 15 minutes of vaccination or had history consistent with anaphylaxis., Conclusions: The vast majority of children (92%) presenting with a potential IgE-mediated AEFI are able to tolerate challenge to a suspect vaccine without reaction. We present our investigation protocol recommending skin testing in all children with anaphylaxis and challenge with a suspect vaccine if negative testing or previous nonanaphylactic potential IgE-mediated AEFI., (Crown Copyright © 2019. Published by Elsevier Inc. All rights reserved.)

This study compared the severity of chronic idiopathic urticaria (CIU) and psychiatric symptoms between patients with different levels of posttraumatic stress disorder (PTSD) and investigated a model depicting the interrelationship between PTSD from past trauma, personality traits, coping strategies, CIU severity and psychiatric symptom severity. One hundred CIU and 60 allergy patients participated in the study, completing measures on PTSD, psychiatric symptoms, personality traits and coping strategies. The results showed that for CIU patients, 7%, 40 and 34% met the diagnostic criteria for no-PTSD, partial-PTSD and full-PTSD respectively whereas for allergy patients, 15%, 45 and 18% met the same criteria. Apart from CIU, psychiatric symptom severity differed significantly between diagnostic groups. PTSD was associated with coping strategies which were in turn associated with CIU severity and psychiatric symptom severity. PTSD was not significantly associated with personality. Emotion-focused coping mediated PTSD and CIU severity, PTSD and psychiatric symptom severity and neuroticism and CIU severity. To conclude, psychiatric symptom severity varies depending on the level of PTSD among CIU patients. Neurotic patients with a high level of PTSD from past trauma show raised CIU and psychiatric symptom severity when using emotion-focused coping strategies.

The present survey represents the latest data on diagnosis and management of childhood urticaria. It has been observed that urticaria occurs less often in children than adults, with symptoms rarely lasting for over 6 weeks. Triggers or aggravating factors can be found only in 21%-55% of cases. Finding autoantibodies in children does not impact a disease prognosis, unlike in adult patients, where the presence of autoantibodies is associated with a more prolonged run of the disease, a more severe prognosis and more intensive treatment methods. The incidence of food allergy equals to 8%-10% of cases. The incidence of Helicobacter Pylori infection in children is lower than that in adults and comes to 10%-18%. Medical experts recommend using the same treatment schemes for adults and children. This survey describes different urticaria management patterns suggested by experts from Europe, America, and Russia in their recent guidelines. It has been noted that unlike the guidelines from 2014, the 2018 clinical practice guidelines for the diagnosis and management of urticarial once again suggest a four-step treatment scheme with assigning omalizumab for Step 3 and cyclosporine A for Step 4 in the event of low therapeutic efficacy of the previous step or its impossibility. Leukotriene antagonists (LTRAs) are currently removed from basic management to alternative programs., (© 2018 EAACI and John Wiley and Sons A/S. Published by John Wiley and Sons Ltd.)

Background: The daily diary Urticaria Activity Score (UAS) and its weekly score (UAS7) are widely used to assess signs and symptoms in patients with chronic spontaneous urticaria (CSU). The objective of this study was to assess the psychometric properties of a Spanish version of the once-daily UAS., Methods: Observational study in patients ≥18 years old receiving usual care for CSU (daily or almost daily occurrence of generalized hives or angioedema for ≥6 weeks). Patients were included consecutively and completed the UAS, EQ-5D, and the Chronic Urticaria Quality of Life scale (CU-Q 2 oL) at two study visits 6 weeks apart. On each occasion, the UAS was completed once-daily for 7 consecutive days to be able to calculate the UAS7 score. Psychometric properties of reliability, construct validity, and responsiveness were assessed. The Minimal Important Difference (MID) was estimated for the UAS7 using anchor- and distribution-based approaches., Results: Data from 166 patients was available for analysis (mean age 49 years, 65.7% female). Floor (5.4% of patients with the lowest possible score) and ceiling (1.2%) effects were low; 15% of patients had missing values. Internal consistency and test-retest reliability were good (Cronbach's alpha of 0.83 and an ICC of 0.84, respectively). Convergent validity was demonstrated through the pattern of correlations with the EQ-5D and CU-Q 2 oL and known groups' validity was demonstrated by the instrument's ability to discriminate between patients with different overall levels of urticaria severity, with between-group effect-sizes (ES) ranging from 0.36 to 1.19. The UAS7 proved responsive to change with effect sizes ranging from 0.3 to 1.52 in patients reporting improvement or deterioration in overall urticaria status. The MID for the UAS7 score was estimated at 7-8 points, on a scale of 0-42., Conclusions: The Spanish version of the UAS score has demonstrated a robust psychometric performance in patients with CSU managed in conditions of usual care. It can therefore be considered a suitable instrument to assess disease activity in clinical practice in Spanish-speaking patients. The Spanish version's reliability and validity are similar to those reported for other language versions of the once- and twice-daily variants of the UAS.

Background: Chronic spontaneous urticaria (CSU) is autoimmune in nature and associated with thyroid autoimmunity (TA), but evidence on autoimmunity in relation to CSU progression and prognosis is limited. We evaluated whether TA and autoimmunity in CSU are correlated with disease severity, therapeutic response, and time to remission and establish an association between CSU characteristics linked to thyroid autoantibody., Methods: Medical records of patients diagnosed with urticaria attending outpatient dermatology clinic at a university-based hospital from 2013 to 2017 were retrospectively reviewed. Data on the clinical characteristics, laboratory investigations particularly thyroid antibody titers, autologous serum skin test (ASST) and autologous plasma skin test (APST) results and their link to disease severity, treatments, and time to remission of CSU patients were analyzed., Results: Of 1,096 patients with urticaria, 60.2% had CSU. Three-hundred patients fulfilled the inclusion criteria for CSU with complete thyroid antibody testing. Positive TA was significantly associated with female gender and age > 35 years (p = 0.008). Antithyroid peroxidase (anti-TPO)-positive patients suffered from CSU longer than 12 and 18 months compared to anti-TPO-negative patients (100.0% vs. 82.6%, p = 0.042, and 100.0% vs. 75.9% p = 0.020, respectively). The presence of urticarial attacks > 4 days/week was significantly seen in ASST and APST-positive patients compared to those without (84.6% vs. 61.3%, p = 0.011, and 85.3% vs. 61.8%, p = 0.006, respectively). Positive APST patients were more difficult to treat than those with negative results (61.2% vs. 37.8%, p = 0.017)., Conclusions: Antithyroid peroxidase is a predictor of time to remission, while autologous skin testing is linked to disease severity (ASST and APST) and therapeutic response (APST) in CSU patients.

Background: Dynamic optical coherence tomography (D-OCT) is a noninvasive imaging technique providing images of the skin and detecting movement in the tissue ie, measuring blood flow. The "attenuation coefficient" describes light absorption and scattering abilities of the tissue, while the dynamic signal provides a quantitative measure of the blood flow., Aim: The study objective is to describe the dynamic changes of the skin and skin vessels during histamine release using D-OCT., Methods: Healthy volunteers had local histamine injections in the skin and D-OCT-scans performed at 2-minute intervals to detect changes in blood flow, attenuation and clinical symptoms., Results: 9/10 participants showed clinical wheals. An increase in blood flow was shown at all depths (P < .001 at 2 minutes). The highest relative increase was seen at 300 μm. The signal at 500 μm decreased to insignificant values and remained low after 4 minutes. A decrease in visualization depth of up to 32.7% as well as a significant increase in the attenuation coefficient was shown (P < .001 at 12 minutes for both tests)., Conclusion: Dynamic optical coherence tomography is able to reliably identify changes in blood flow of histamine induced wheals. Dermal oedema reduces visualization depth and increases the attenuation coefficient., (© 2018 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Chronic spontaneous urticaria (CSU) is a common itchy skin disease. Despite its prevalence, the neuropathology of CSU is uncertain. In this study, we explored resting state functional connectivity (rs-FC) changes in CSU, as well as how the symptom changes following intervention can modulate rs-FC. Forty patients and 40 healthy controls (HCs) were recruited. Following an intervention, 32 patients participated in a second scan approximately 6 weeks after the first scan. Compared with healthy controls, CSU subjects exhibited higher regional homogeneity (ReHo) values in the cerebellum, which were positively associated with urticaria activity scores over 7 days (UAS7) at baseline. After an intervention accompanied with clinical improvement, we found that ReHo values decreased at the cerebellum and increased at the bilateral primary somatosensory cortex (SI)/primary motor cortex (MI)/supplementary motor area (SMA). Using the cerebellum as a seed, CSU subjects exhibited increased rs-FC with reward regions when compared with HCs and exhibited decreased rs-FC at the right orbitofrontal cortex and right sensorimotor region following the intervention. The improvement rate values were positively associated with reduced rs-FC values in the two regions. Using the cluster of SI/MI/SMA as a seed, CSU patients exhibited decreased rs-FC with the left putamen, caudate, accumbens, and thalamus following the intervention. These results demonstrate the altered cerebellar activity and cerebellum-reward-sensorimotor loops in CSU.

Little attention has been given to the burden of chronic urticaria (CU) in Japan compared with other skin diseases, such as atopic dermatitis (AD) and psoriasis. The primary objective of the RELEASE study was to evaluate the real-life quality-of-life impairment in CU patients in Japan. Data were collected from 1443 urticaria, 1668 AD and 435 psoriatic patients; 552 urticaria patients who presented urticaria symptoms for over 6 weeks were defined as CU. The mean Dermatology Life Quality Index (DLQI) total score was 4.8, 6.1 and 4.8 in CU, AD and psoriatic patients, respectively. Disease control of urticaria evaluated by the Urticaria Control Test (UCT) and DLQI exhibited a strong correlation with a Spearman's rank correlation coefficient of -0.7158. CU and AD patients had relatively higher scores in all Work Productivity and Activity Impairment - General Health subscales except for absenteeism. At the time of the survey, approximately 64% of CU patients reported UCT scores of <12 and demonstrated higher work productivity loss and activity impairment versus patients with UCT scores of ≥12. Patients with lower UCT scores also displayed a higher percentage of dissatisfaction with their health state and the treatment they received. Approximately 85% of patients with CU had visited dermatology clinics, and less than 20% had visited hospital, indicating existence of a highly burdened population outside specialized centers. These results highlight the unmet medical needs of CU patients, suggesting the need to increase awareness of CU burden among both physicians and patients and to pursue improved real-life patient care., (© 2018 Novartis K.K. The Journal of Dermatology published by John Wiley & Sons Australia, Ltd on behalf of Japanese Dermatological Association.)

Background: Solar urticaria (SU) is a rare chronic inducible urticaria triggered via uncharacterized chromophores. We detail responses of a large patient series to monochromator phototesting and broadband ultraviolet radiation (UVR); relationship to life quality is explored., Methods: Retrospective review of all SU patients undergoing standardized diagnostic photoinvestigation at a specialist centre during 2000-2016. From 2011, patients completed dermatology life quality index (DLQI) questionnaires for the past week and year., Results: In 145 patients (mean: 35.8, range: 3-69 years; 18 aged <18 years; 100 female), combined phototesting with broadband UVR and monochromator sources successfully provoked 74.5% patients, with 65.6% provoked by broadband UVR alone and 57.9% by monochromated radiation alone. The narrow wavebands most frequently eliciting wheal and flare response were between 370 and 400 nm, with 25% patients at 300 ± 5 nm, 53.6% at 320 ± 10 nm, 66.7% at 330 ± 10 nm, 77.4% at 350 ± 20 nm, 83.3% at 370 ± 20 nm, 86.9% at 400 ± 20 nm, 44% at 500 ± 20 nm and 17.8% at 600 ± 20 nm. In 62 patients, the DLQI revealed 56.1% had very to extremely large impact in the past week (all patients: mean score: 11.1, range: 0-29) rising to 69.8% for the past year (12.5, 0-30); adults and children were similarly affected. Patients with positive photoprovocation had higher DLQI score than those who were negative (DLQI for past week: mean: 12.6 ± SEM 1.1 vs 4.6 ± 1.4, P < .01)., Conclusion: SU is predominantly provoked by longer UVA-shorter visible radiation, which penetrates window-glass and where sunscreens are less effective; impact on life quality is considerable. Photoprotective agents effective against this spectrum are needed., (© 2018 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Objective: This article reviews current findings regarding the management of stress in allergic disease., Data Sources: The authors use articles and books published between 1995 and 2017. Approximately 85% of sources used were published in the last 10 years, and 60% were published in the last 5 years. Most of the sources are peer-reviewed articles., Study Selections: Articles that focused on allergic diseases such as allergic rhinitis, food allergies, urticaria, and allergic asthma were included. Articles in which whether the underlying disorder was allergic in nature (for example, nonspecified asthma) were not included. Preference was given to articles published within the past five years., Results: Patients with allergic diseases, particularly those with chronic or co-occurring allergic diseases, often experience stress and, in turn, this experience of stress can exacerbate disease presentation. High rates of treatment nonadherence in patients with allergic disease also can increase disease burden and stress. Research supports the benefit of behavioral health interventions for patients with allergic disease. Interventions with multidisciplinary teams, which include behavioral health, as well as interventions at the school, workplace, and community level, are recommended., Conclusion: Medical providers working with patients with allergic disease need to address patients' experience of stress and nonadherence to treatment recommendations. This could be done through routine screening and referrals to behavioral health or, ideally, through incorporation of a behavioral health provider within a multidisciplinary patient team., (Copyright © 2018 American College of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.)

Background: Chronic spontaneous urticaria (CSU), also known as chronic idiopathic urticaria, may produce hives, itch, and angioedema. The Urticaria Activity and Impact Measure (U-AIM) is a newly developed 9-item patient-reported measure designed for use in routine clinical practice to assess CSU activity and impact during the previous 7 days., Objective: To evaluate validity, responsiveness, and clinically meaningful change of the U-AIM., Methods: Data from a 24-week, open-label, single-arm period of a randomized, placebo-controlled study of omalizumab were used to assess the psychometric properties of U-AIM items for itch, hives, and angioedema., Results: A total of 206 patients (75% female; mean age, 44.6 years) were enrolled. At baseline, U-AIM results included prevalent severe itch (55%) and more than 12 hives (67%), angioedema (15%), and bother by itch (84%), hives (84%), and angioedema (49%). The Urticaria Patient Daily Diary (UPDD) mean weekly scores were 15.4 (itch severity), 16.8 (number of hives), and 32.2 (Urticaria Activity Score [UAS7]). At baseline, week 12, and week 24, U-AIM itch and hives items and UAS7 proxy scores (the sum of itch severity and number of hives during 7 days) demonstrated strong correlation coefficients with their corresponding measures from the UPDD (itch severity: 0.634-0.806; hives number: 0.735-0.843; UAS7 proxy: 0.724-0.852). Changes in U-AIM scores differentiated patients by their perspective of symptom improvement. Meaningful change thresholds were established for itch severity and number of hives scores (range, 0.8-1.0 for both) and the UAS7 proxy score (range, 10.5-12.5)., Conclusion: The U-AIM is valid and responsive to change and may help clinicians monitor CSU activity and track treatment effectiveness., (Copyright © 2018 The Authors. Published by Elsevier Inc. All rights reserved.)

Background: Mycoprotein, which is produced by a mold and is the basis of Quorn-brand meat substitutes, is a novel cause of allergic and gastrointestinal reactions, but little information has been available on its associated symptomatology., Objective: To describe the nature and frequency of adverse reactions to mycoprotein., Methods: Self-reports of adverse reactions to mycoprotein were collected via a Web-based questionnaire (www.quorncomplaints.org) and then analyzed., Results: Analysis of 1,752 adverse reactions found that Quorn products caused allergic and gastrointestinal symptoms, with some people experiencing both. Allergic reactions, including urticaria and anaphylaxis, occurred within 4 hours of consumption in 312 people. Of those reactions, 45.8%, 1 fatal, began within 1 hour of exposure. Of those 312 individuals, 188 (60.3%) reported repeated reactions after repeated consumption of Quorn, and 2 people experienced 8 reactions (13 people did not say whether they experienced more than 1 reaction). Quorn foods caused gastrointestinal symptoms, including emesis and diarrhea, within 8 hours of consumption in 1,692 people. Of the gastrointestinal symptoms, 66.6% occurred 46 to 180 minutes after consumption of the products. Symptoms ranged from mild nausea to emesis severe enough to warrant medical attention., Conclusion: Mycoprotein may be causing numerous and sometimes life-threatening allergic and gastrointestinal reactions. The acceptance in the food supply of this nonessential ingredient deserves reconsideration., (Copyright © 2018 American College of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.)

Chronic spontaneous urticaria (CSU) is a common skin disorder characterized by daily or almost daily recurring skin edema and flare with itch. Recently, the activation of the blood coagulation cascade has been suggested to be involved in CSU, but the trigger of the coagulation cascade remains unclear. In this article, we review recent understanding of the relationship between the pathogenesis of CSU and extrinsic coagulation reactions. In CSU, vascular endothelial cells and eosinophils may play a role as TF-expressing cells for activating the extrinsic coagulation pathway. Moreover, the expression of TF on endothelial cells is synergistically enhanced by the activation of Toll-like receptors and histamine H 1 receptors. The activated coagulation factors may induce plasma extravasation followed by degranulation of skin mast cells and edema formation recognized as wheal in CSU. Molecules involved in this cascade could be a target for new and more effective treatments of urticaria., (Copyright © 2017 Japanese Society of Allergology. Production and hosting by Elsevier B.V. All rights reserved.)

Purpose: The aim of this study was to review the evidence on the epidemiology, physiopathology, categorization, and management of cholinergic urticaria. We specifically focused on several subtypes of cholinergic urticaria and investigated the relationship between cholinergic urticaria and idiopathic anhidrosis., Methods: Using an integrative approach, we reviewed publications addressing the epidemiology, clinical features, diagnostic approach, physiopathology, subtype classification, and therapeutic approach to cholinergic urticaria., Results: Multiple mechanisms were found to contribute to the development of cholinergic urticaria. This disorder should be classified based on the pathogenesis and clinical characteristics of each subtype. Such a classification system would lead to better management of this resistant condition. In particular, sweating function should be given more attention when examining patients with cholinergic urticaria., Conclusions: Because cholinergic urticaria is not a homogeneous disease, its subtype classification is essential for selection of the most suitable therapeutic method.

Chronic spontaneous urticaria (CSU) is associated with activation of acute phase response. Questions arise regarding its association with other inflammatory mediators. To determine plasma IL-8 concentration in CSU patients and its association with C-reactive protein (CRP) concentration, a nonspecific inflammatory marker of the disease activity, concentrations of plasma IL-8 and serum CRP were measured in CSU patients and compared with healthy controls. IL-8 and CRP concentrations were significantly higher in CSU patients as compared with the healthy subjects. In addition, there were significant differences in IL-8 and CRP concentrations between CSU patients with moderate-severe symptoms and the healthy subjects. Plasma IL-8 and serum CRP concentrations showed a significant correlation with urticaria activity score (UAS). Additionally, a significant positive correlation was observed between IL-8 and CRP concentrations. Up-regulations of IL-8 and its association with the marker of clinical and inflammatory activity suggest a role of this cytokine in the pathogenesis of CSU.

Background: Chronic spontaneous/idiopathic urticaria (CSU/CIU) has substantial detrimental effects on health-related quality of life (HRQoL) with an effect comparable to or worse than many other skin diseases., Objective: To assess the effect of omalizumab on CSU patients' HRQoL, measured by the Dermatology Life Quality Index (DLQI) in three phase III studies ASTERIA I, ASTERIA II and GLACIAL., Methods: A post hoc analysis examined changes in DLQI scores, distribution of patients across DLQI bands and the proportion reaching minimal clinically important difference (MCID) following omalizumab vs. placebo., Results: Omalizumab 300 mg significantly improved total DLQI scores vs. placebo, with a mean decrease from baseline to week 12 of -10.3 vs. -6.1 (P < 0.0001) in ASTERIA I, -10.2 vs. -6.1 (P = 0.0004) in ASTERIA II and -9.7 vs. -5.1 (P < 0.0001) in GLACIAL. A significant shift from high disease impact on life at baseline towards less impact at week 12 was seen with omalizumab 300 mg vs. placebo (P < 0.001; all studies). The proportion of patients where change in mean total DLQI score from baseline to week 12 reached an MCID of ≥4 was 74.1%, 76.0% and 77.2% in ASTERIA I, II and GLACIAL, respectively (P < 0.01; all studies)., Limitations: Maximum duration of omalizumab treatment was 24 weeks., Conclusion: This additional analysis assessed the impact of CSU and benefit of treatment with omalizumab by exploring different facets of DLQI data by treatment arm at multiple assessment points. The original aspects of analysis included applying the concept of the recently validated score for the MCID of the DLQI, changes in DLQI domain scores and in the distribution of subjects based on validated total DLQI score bands. It showed consistently that omalizumab provides significant and clinically relevant improvements in many aspects of HRQoL that are important to patients with CSU. These results contribute to a better understanding of the impact of CSU and its treatment on patients and can support clinical decision-making in routine medical practice., (© 2017 The Authors. Journal of the European Academy of Dermatology and Venereology published by John Wiley & Sons Ltd on behalf of European Academy of Dermatology and Venereology.)

Background: Patients with chronic idiopathic urticaria (CIU)/chronic spontaneous urticaria sometimes report systemic complaints (SCs)., Objective: We sought to determine the frequency and characteristics of SCs among patients with CIU, as well as the association of SCs with disease measures, basophil histamine release, and serum tryptase., Methods: Adult patients with CIU were recruited from a university allergy clinic. Patients completed a disease symptom survey and underwent blood sampling for subsequent basophil histamine release and serum tryptase measurement., Results: A total of 155 patients with CIU were surveyed, with 103 reporting SCs with concomitant hives as follows: joint pain or swelling (55.3%), headache/fatigue (47.6%), flushing (42.7%), wheezing (30.1%), gastrointestinal complaints (26.2%), and palpitations (9.7%). Patients with SCs (CIU-SC) were compared with those with no SCs (CIU-NSC). Both groups had similar demographic characteristics (average age in 40s, majority female and white) and basophil histamine release profiles. CIU-SC had significantly greater disease duration (51.5% CIU-SC vs 30.8% CIU-NSC had >4 years duration), emergency department visits (41.7% vs 23.1% had >1 visit in the last year), CIU-related work absences (65% vs 27.5% had >1 day), oral corticosteroid use (84.5% vs 59.6%), quality-of-life impairment (76.1 vs 59.2 SkinDex score), and serum tryptase levels (5.1 ng/mL vs 3.9 ng/mL)., Conclusions: Despite similar demographic characteristics and basophil profiles as patients with CIU-NSC, patients with CIU-SC have features of greater disease burden (work absences, emergency department visits, and corticosteroid use), quality-of-life impairment, and baseline serum tryptase levels., (Copyright © 2017 American Academy of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.)

Background: Approximately 50% of patients with chronic idiopathic/spontaneous urticaria (CIU/CSU) report hives and angioedema; some experience hives/angioedema only., Objective: Assess omalizumab's effect on angioedema and quality of life (QoL) in subgroups with refractory CIU/CSU: those with and without angioedema., Methods: Patients received omalizumab (75, 150 or 300 mg) or placebo every 4 weeks for 12/24 weeks. Angioedema and QoL were assessed [Urticaria Patient Daily Diary and Dermatology Quality of Life Index (DLQI)]. Subgroups were based on the presence/absence of baseline angioedema 7 days prior to randomization., Results: Patients with baseline angioedema randomized to omalizumab 300 mg had a greater reduction in mean weekly incidence of angioedema and mean number of days/week with angioedema vs. placebo at 12 and 24 weeks. A 3.3- to 4.5-point greater mean reduction in DLQI score was achieved with omalizumab 300 mg treatment vs. placebo, above the minimal clinically important difference threshold. Results with lower doses vs. placebo were variable., Conclusion: Compared with placebo, omalizumab 300 mg treatment over 12-24 weeks resulted in marked reduction in incidence and number of days/week with angioedema accompanied by clinically relevant improvement in QoL., (© 2016 European Academy of Dermatology and Venereology.)

Background: Chronic urticaria (CU) is characterized by the recurrence of itchy hives and/or angioedema for more than 6 weeks. AWARE (A World-wide Antihistamine-Refractory Chronic Urticaria Patient Evaluation) is a multinational study designed to document the real-life treatment situation, burden of disease and clinical resource usage of H1-antihistamine-refractory CU patients., Objective: To examine baseline data from Scandinavian AWARE patients., Methods: AWARE is a prospective, non-interventional, multinational, umbrella design study, which includes adults (≥18 years) with a confirmed CU diagnosis (>2 months) that is refractory to H1-antihistamines. Baseline patient characteristics, disease activity (urticaria control test [UCT]), pharmacological treatment, comorbidities and healthcare usage were documented by the treating physician. Quality of life (QoL; dermatology life quality index [DLQI]; chronic urticaria quality of life questionnaire [CU-Q 2 oL; Danish patients only]) and work productivity and activity impairment (WPAI) scores were also assessed., Results: Overall, 158 CU patients from seven centres in Denmark (n = 80), Norway (n = 50) and Sweden (n = 28) were included in this baseline analysis. Mean age and BMI were 40.3 years and 26.5 kg/m 2 , respectively. The majority of patients were female (69.6%), had uncontrolled CU (75.6%; UCT score <12) and had a 'spontaneous' component to their CU (61.4% CSU; 20.3% both CSU and chronic inducible urticaria). Common comorbidities included asthma (19.6%), allergic rhinitis (16.5%) and food allergies (8.2%). Overall, 60.1% of patients reported using treatments for CU including non-sedative H1-antihistamines (40.5%), corticosteroids (19%), montelukast (14.6%) and omalizumab (8.2%). Pharmacological treatment rates increased to 96.2% during the baseline visit. On average, patient QoL was moderately affected (mean DLQI score 7.7) and healthcare resource usage was high., Conclusion: Adult Scandinavian H1-antihistamine-refractory CU patients reported high rates of healthcare usage and QoL impairment. Rates of pharmacological treatment use were low before study enrolment but increased to almost 100% during the baseline visit., (© 2017 European Academy of Dermatology and Venereology.)

Background: There is little information regarding the etiology and natural course of chronic spontaneous urticaria (CSU) in childhood., Objective: To investigate the etiology, prognosis, and the factors associated with the prognosis of CSU in children., Method: Data from children with CSU who had been diagnosed between 1992 and 2015 were analyzed. A telephone interview was done to assess the current status of these patients. Remission was defined as the disappearance of urticaria for >6 months., Results: A total of 222 children with CSU were evaluated. The median age of symptom onset was 8.8 years (interquartile range [IQR], 4.6-12.3 years), median duration of urticaria was 23 months (IQR, 7-48 months), and the median sum of the daily urticaria activity score of 7 consecutive days (UAS7) was 28 (IQR, 21-42). Accompanying angioedema was reported by 107 patients (48.2%), whereas 27.1% of the study population had autoantibody positivity. Autologous serum skin testing results were positive in 43 (34.1%); skin-prick testing results revealed atopy in 55 children (27.9%). Parasites (4.8%), pollen sensitization (1.5%), food allergy (0.9%), urinary tract infection (0.9%), and Hashimoto thyroiditis (0.5%) were determined as etiologic factors of CSU. The patients were followed up for a median time of 15 months (IQR, 5-36.5 months). Remission was observed in 10.6, 29.3, and 44.5% of the patients in 1, 3, and 5 years, respectively. In multivariate regression analysis, a UAS7 of >28 at admission was found to be a risk factor for persistence of urticaria (odds ratio 6.22 [95% confidence interval, 1.54-25.15; p = 0.010)., Conclusion: The etiology of CSU in children was mostly idiopathic despite detailed investigation. In childhood, the natural course of CSU was favorable, and nearly half of the patients recovered after 5 years of disease duration. A high UAS7 at admission seemed to be a significant risk factor for the persistence of symptoms.

Objective: To explore the association among C reactive protein (CRP) level, disease severity and non-sedating antihistamine drug efficacy in patients with chronic spontaneous urticaria.
 Methods: A total of 605 patients with chronic spontaneous urticaria from August 2013 to March 2015, diagnosed by dermatologist of Xiangya Hospital according to the guideline, were enrolled in present study. The patients were divided into two groups according to the weekly urticaria activity score (UAS): a response group (more than 50% reduction) and a nonresponse group (less than 50% reduction). T test and Pearson correlation analysis were used to analyze the correlation between the clinical characteristics (such as disease severity, drug efficacy) and CRP in patients with chronic spontaneous urticaria.
 Results: The CRP levels were significantly higher in patients with severe chronic spontaneous urticaria as compared to those with moderate chronic spontaneous urticaria (t=-2.715, P<0.01). Meanwhile, after treatment with secondary antihistamine drug, the patients with lower CRP levels showed better responses than those with higher CRP levels [(2.5764±2.5059) and (3.6715±4.7732) mg/dL respectively; t=-2.187, P<0.05].
 Conclusion: Serum CRP level before the treatment may be correlated with the severity of chronic spontaneous urticaria and efficacy of treatment.

Diseases characterized by recurrent symptoms with prolonged intervals without any clinical manifestations can pose diagnostic difficulties. Some diagnoses will be obvious but other situations can be very challenging.To nosologically delineate a new entity characterized by recurrent flares of induration of the forearms and legs with swelling of the extremities accompanied by intense fatigue and variable other symptoms.Retrospective observational study of patients recorded from 2000 to 2015. All patients included were seen during a consultation at the Dermatology Department of the University Hospital of Strasbourg, France. We retrieved the medical records from patients seen and recorded over the last 16 years having induration of the extremities, the forearm and the legs occurring between 4 and 12 hours after a physical effort accompanied by systemic signs that lasted for a few days. We analyzed in detail the clinical and biological features, evolution, and treatments of these patients.We included 6 males, with a mean age of 47 years; mean age at disease onset was 42. All patients were initially misdiagnosed as having rheumatic disorders. The mean delay before diagnosis was 5 years. The main complaint was painful induration or muscle soreness of the forearms and the legs associated with transient functional impairment and prolonged asthenia for a mean duration of 3.5 days. Induration of the deep soft tissues was very suggestive of myofasciitis. The delay between the triggering physical effort and the swelling was between 6 and 12 hours. Physical effort as triggering factor was never spontaneously mentioned. Two patients had partial response to high dose antihistamines and 2 other patients to the interleukin-1 inhibitor anakinra. One patient responded to hydroxychloroquine.The very stereotypical presentation in those 6 patients suggests that this is a recognizable entity characterized by effort-induced induration of forearms and/or legs, due to deep edematous myofascial involvement, occurring a few hours after a physical effort. We suggest to name this entity delayed effort-induced swelling with myofasciitis and systemic manifestations.

Introduction: chronic urticaria (CU) is a skin disorder characterized by transient, pruritic wheals persisting for longer than 6 weeks. The etiopathogenesis of the disease is still unclear, but there is evidence that autoimmunity and endocrine dysfunction may be involved., Aim: the aim of this study was to determine whether chronic urticaria is statistically associated with thyroid autoimmunity., Patients and Methods: in a prospective case-control study, we compared the frequency of thyroid auto-antibodies (thyroglobulin antibody, anti-Tg and thyroid peroxidase antibody, anti-TPO) in 70 patients with chronic urticaria and in 70 healthy volunteers. Thyroid auto-antibodies and thyroid hormones (thyroxine (T4), triiodthyronine (T3) and thyroid stimulating hormone (TSH) were measured in all subjects., Results: thyroid functional abnormalities were found in 8 (11.43%) patients. Anti-Tg and anti-TPO were positive in 16 (23%) and 21 (30%) patients, respectively. In control group, only one subject (1.42%) had abnormalities in thyroid hormonal status, and two subjects (2.86%) had positive thyroid auto-antibodies. Compared with the control group, the frequency of both anti-Tg and anti-TPO was significantly higher in those with chronic urticaria (P < 0.05)., Conclusion: this study shows a significant association between chronic urticaria and thyroid autoimmunity, and that tests to detect thyroid auto-antibodies are relevant in patients with chronic urticaria., Competing Interests: • Conflict of interest: none declared.

Background: Cholinergic urticaria (CholU) is a frequent chronic urticaria disorder with itchy weal and flare-type skin reactions in response to physical exercise or passive warming. A higher frequency of atopy among CholU patients has been reported, but the significance of this observation is unclear., Objective: To assess the prevalence and relevance of atopy in CholU patients., Materials and Methods: Thirty CholU patients were assessed for atopic skin diathesis (atopic predisposition) by use of the Erlangen Atopy Score and divided into atopic and non-atopic predisposed CholU individuals. Both groups were assessed for disease severity (CholUSI) and activity (CholUAS7), quality of life impairment [Dermatology Life Quality Index (DLQI) and CU-Q 2 OL], seasonal exacerbation, total and specific serum IgE and comorbidities., Results: CholU patients were found to exhibit high rates of atopic predisposition (57%), with higher prevalence and scores in female than in male patients. High Erlangen Atopy Scores were linked to high CholU severity, activity and impact on QoL. Atopic predisposed CholU patients show different seasonal exacerbation patterns, IgE specificity and comorbidity profiles as compared to non-atopic CholU patients., Conclusion: Atopic predisposition and cholinergic urticaria appear to be linked more closely than previously thought, which suggests shared pathogenetic mechanisms. Atopic patients with cholinergic urticaria have more severe disease and poorer quality of life than those who do not. Thus, all cholinergic urticaria patients should be assessed for atopic predisposition., (© 2016 European Academy of Dermatology and Venereology.)

Introduction : Anaphylaxis is a predominantly childhood disease. Most of the literature on anaphylaxis has emerged from Western countries. This study aimed to describe the incidence, triggers and clinical presentation of anaphylaxis among children in Singapore, look for predictors for anaphylaxis with severe outcomes, and study the incidence of biphasic reactions. Materials and Methods : We retrospectively reviewed records of children presenting with anaphylaxis to our paediatric emergency department from 1 January 2007 to 31 December 2014. Results : We identified 485 cases of anaphylaxis in 445 patients. Cutaneous symptoms (urticarial/angio-oedema) were the most common across all age groups (481 cases, 99%), followed by respiratory (412, 85%), gastrointestinal (118, 24%) and cardiovascular (35, 7.2%) symptoms. Central nervous system symptoms (drowsiness/ irritability) were rare across all age groups (11, 2.2%). Food was identified as the most common trigger across all age groups (45% to 63%). Seafood was the most common food trigger (57, 25%). A total of 420 (86.6%) children were treated with adrenaline, 451 (93%) received steroids and 411 (85%) received antihistamines. Sixty-three (13%) children fulfilled the criteria of severe anaphylaxis. There was no statistically significant association between severe anaphylaxis and the type of trigger ( P = 0.851), nor an overall past history of atopy ( P = 0.428). The only independent predictor for severe anaphylaxis was a previous drug allergy ( P = 0.016). A very low prevalence of biphasic reactions (0.6% of study population) was noted in our study. Conclusion : We described the presentation and management of anaphylaxis in the Singapore population. A history of drug allergy is associated with severe presentation. Biphasic reactions are rare in our population.