Aquagenic urticaria in twins

We describe the case of 18 year old twin brothers who presented to our unit with a 3 year history of aquagenic urticaria. This rare form of urticaria usually presents within an hour of contact with water. The aetiology is unknown. Most cases are sporadic but there are a small number of familial cases in the medical literature. A specific genetic mutation has not yet been found. To our knowledge, this is the first report of aquagenic urticaria in monozygotic twins, further supporting a genetic component to this disease

Helminths and allergic disease in Vietnam

Background: Allergic disease is uncommon in developing countries, especially in rural areas. A protective effect of helminth infection has been implicated as a potential explanation. Objectives: To determine whether reduced exposure to helminth infection is associated with a higher risk of allergen skin sensitisation and allergic disease, and whether such an association could be explained by a helminth-induced up-regulation of certain cytokines, in particular anti-inflammatory IL-10. Methods: We invited 1,742 rural Vietnamese schoolchildren to take part in a cross-sectional baseline survey followed by a randomised, double blind, placebo-controlled trial of anti-helminthic therapy at 0, 3, 6, and 9 months to compare the change in exercise-induced bronchospasm (primary outcome), wheeze, rhinitis, eczema, and allergen skin sensitisation (secondary outcomes) at 12 months. 244 secondary schoolchildren also had venous blood taken to measure helminth induced IL-10, IFN-gamma, IL-5, and IL-13. Out of these 244 children, 144 were infected with hookworm and had bloods taken again at 12 months. Results: Baseline survey 1,601 schoolchildren (92% of those eligible) in grades 1-9 aged 6-18 participated in the baseline survey. 0.4% (6/1601) of children had a fall in peak flow after exercise of at least 15%. Doctor-diagnosed asthma was equally rare (0.4%, 6/1601), while 5.0% (80/1601) of children had experienced wheezing over the past 12 months. 6.9% (110/1601) of parents reported that their children had suffered of hay fever in the past 12 months, and in 2.6% (41/1601) of cases this diagnosis was confirmed by a doctor. 5.6% of children (89/1601) reported an itchy rash over the past 12 months. 0.9% (14/1601) had a history of flexural involvement and on examination 0.5% (8/1601) proved to have flexural eczema on the day of the survey. Skin prick test positivity was commoner than allergic disease. 33.5% (537/1601) of children had at least one positive skin prick test (dustmites 14.4%, cockroach 27.6%). The cross-sectional analysis yielded only significant results for allergen skin sensitisation. In univariate analysis, sensitisation was less frequent in children with hookworm or Ascaris infection, and increased in those with better santitation, including flush toilets and piped drinking water. In multivariate analysis, the risk of allergen skin sensitisation to house dust mite was reduced in those with Ascaris lumbricoides infection (adjusted OR=0.28, 95% CI 0.10-0.78) and in children with higher hookworm burden (adjusted OR for 350+ versus no eggs per gram faeces=0.61, 0.39-0.96), and increased in those using flush toilets (adjusted OR for flush toilet versus none/bush/pit=2.51, 1.00-6.28). In contrast, sensitisation to cockroach was not independently related to helminth infection but was increased in those regularly drinking piped or well water rather than from a stream (adjusted OR=1.33, 1.02-1.75). Intervention study 1,566 children in grades 1-8 completed the baseline survey and all consented to be randomised to either anti-helminthic treatment or placebo. 1487 children (95%) completed the intervention study. There was no effect of therapy on the primary outcome, exercise-induced bronchoconstriction (within-participant mean % fall in peak flow from baseline after anti-helminthic treatment 2.25 (SD 7.3) vs placebo 2.19 (SD 7.8, mean difference 0.06 (95% CI -0.71-0.83), p=0.9), or on the prevalence of the secondary clinical outcomes questionnaire-reported wheeze (adjusted OR=1.16, 0.35-3.82), rhinitis (adjusted OR= 1.39, 0.89-2.15), or flexural eczema (adjusted OR=1.17, 0.39-3.49). However, anti-helmithic therapy was associated with a significant allergen skin sensitisation risk increase in the treatment compared to the placebo group (adjusted OR=1.31, 1.02-1.67). In post-hoc analysis this effect was particularly strong for children infected with Ascaris lumbricoides at baseline (adjusted OR=4.90, 1.48-16.19), the majority of whom were co-infected with hookworm. Cytokine profiles Hookworm-induced IL-10 was inversely related to allergen skin sensitisation (any positive skin prick test) at baseline, but this result missed conventional statistical significance (univariate OR=0.70, 0.48-1.03; adjusted OR=0.72, 0.44-1.18). No other cytokine response was associated with skin prick test positivity at baseline (univariate OR IFN-gamma=1.15, 0.71-1.85; univariate OR IL-5=0.84, 0.53-1.33). Similary, no significant changes in any of the cytokine profiles were observed following anti-helminthic therapy in the treatment compared to the placebo group (p=0.3 for all three cytokines). Conclusion The baseline study suggested that hookworm and Ascaris infection, sanitation and water supply independently reduce the risk of allergic sensitisation. The intervention study confirmed that helminth infection and allergic sensitisation are inversely related and that the effect of Ascaris and hookworm infections on skin prick test responses is additive. However, we found little evidence to suggest that this effect was mediated by IL-10. There was also insufficient evidence to suggest that loss of exposure to gut worms for 12 months results in an increase in clinical allergic disease. The effect of more prolonged de-worming warrants further research

How epidemiology has challenged 3 prevailing concepts about atopic dermatitis

We challenge 3 prevailing concepts in understanding atopicdermatitis using data from epidemiologic studies. First, weshow that although atopy is associated with atopic dermatitis tosome degree, its importance is not likely to be a simple causeand-effect relationship, especially at a population level. Ourepidemiologic data do not exclude a contributory role for IgEmediatedimmunologic processes, especially in those withexisting and severe disease. Second, evidence is presented thatdoes not support a straightforward inverse relationshipbetween infections and atopic dermatitis risk. A link, if present,is likely to be more complex, depending critically on the timingand type of infectious exposure. Third, recent evidence suggeststhat the risk of subsequent childhood asthma is not increased inchildren with early atopic dermatitis who are not also earlywheezers, suggesting a comanifestation of phenotypes ratherthan a progressive atopic march. Collectively, theseobservations underline the importance of epidemiologic studiesconducted at a population level to gain a more balancedunderstanding of the enigma of atopic dermatitis

The Role of Topical Timolol in the Treatment of Infantile Hemangiomas: A Systematic Review and Meta-analysis.

To date the efficacy and safety of topical timolol in the treatment of infantile hemangioma has not been reviewed and analysed systematically. We collated all published data on the efficacy and safety of topical timolol in the treatment of infantile hemangioma. A total of 31 studies with 691 patients were included. The fixed effects pooled estimate of the response rate defined as any improvement from baseline of infantile hemangioma after treatment with topical timolol was significant (RR = 8.96; 95% CI 5.07-15.47; heterogeneity test p = 0.99), and the treatment was overall well tolerated. However, the quality of evidence was low to moderate. Topical timolol is an effective treatment for small infantile hemangioma, with no significant adverse effects noted. However, there is still a need for adequately powered randomised controlled trials

Fermionic Expressions for the Characters of c(p,1) Logarithmic Conformal Field Theories

We present fermionic quasi-particle sum representations consisting of a single fundamental fermionic form for all characters of the logarithmic conformal field theory models with central charge c(p,1), p>=2, and suggest a physical interpretation. We also show that it is possible to correctly extract dilogarithm identities.Comment: 19 pages, LaTeX; v2: typos corrected. This is the version accepted for publication in Nucl. Phys.

Measurement properties of quality-of-life measurement instruments for infants, children and adolescents with eczema: a systematic review

Background: Quality of life (QoL) is one of the core outcome domains identified by the Harmonising Outcome Measures for Eczema (HOME) initiative to be assessed in every eczema trial. There is uncertainty about the most appropriate QoL instrument to measure this domain in infants, children and adolescents. Objectives: To systematically evaluate the measurement properties of existing measurement instruments developed and/or validated for the measurement of QoL in infants, children and adolescents with eczema. Methods: A systematic literature search in PubMed and EMBASE, complemented by a thorough hand search of reference lists, retrieved studies on measurement properties of eczema QoL instruments for infants, children and adolescents. For all eligible studies, we judged the adequacy of the measurement properties and the methodological study quality with the COnsensus-based Standards for the selection of health Measurement INstruments (COSMIN) checklist. Results from different studies were summarized in a best evidence synthesis and formed the basis to assign four degrees of recommendation. Results: 17 articles, 3 of which were found by hand search, were included. These 17 articles reported on 24 instruments. No instrument can be recommended for use in all eczema trials because none fulfilled all required adequacy criteria. With adequate internal consistency, reliability and hypothesis testing, the US version of the Childhood Atopic Dermatitis Impact Scale (CADIS), a proxy-reported instrument, has the potential to be recommended depending on the results of further validation studies. All other instruments, including all self-reported ones, lacked significant validation data. Conclusions: Currently, no QoL instrument for infants, children and adolescents with eczema can be highly recommended. Future validation research should primarily focus on the CADIS, but also attempt to broaden the evidence base for the validity of self-reported instruments

How "benign" is cutaneous mastocytosis? A Danish registry-based matched cohort study.

BACKGROUND: There are limited estimates of the incidence rates (IRs) of mastocytosis, and only a few studies have addressed the long-term consequences of living with these diagnoses. Previous reports have shown that systemic mastocytosis is associated with leukemic transformations and an increased risk of death as opposed to cutaneous mastocytosis (CM) and indolent systemic mastocytosis (ISM), which have benign diagnoses with life expectancy rates similar to those of the background population. OBJECTIVE: This study aimed to analyze the incidence and mortality of mastocytosis. METHODS: A population-based matched cohort study of patients with mastocytosis between 1 January 1, 1977 and 31 December 31, 2014 was identified from the Danish National Health Registries. IRs of CM, ISM, and pediatric mastocytosis were highlighted. Survival estimates were compared with those of a healthy background population, using a Cox proportional hazard model. RESULTS: A total of 1461 patients with mastocytosis were identified. The annual IR of overall mastocytosis was 1.1 per 100,000 person years (95% confidence interval [CI], 1.0-1.2). Among children, the IR was 1.8 per 100,000 person years (95% CI, 1.6-2.1). The prevalence of any comorbidity was twice as high among patients with mastocytosis compared with the population without mastocytosis (odds ratio: 2.1; 95% CI, 1.8-2.5). The Charlson Comorbidity Index-adjusted mortality among adult patients with mastocytosis was HRCutaneous Mastocytosis 1.2 (95% CI, 0.8-1.9), HRIndolent Systemic Mastocytosis 1.9 (95% CI 1.4-2.5), and HRSystemic Mastocytosis 4.2 (95%, CI 1.9-9.4), respectively. CONCLUSION: Based on an entire nation, with free health care at the point of access, we estimated an annual IR of mastocytosis and its subgroups. We discovered that patients with ISM had an increased risk of death compared with the general population. Our data supported the overall benign nature of CM diagnosed after age 2 years

The future is now: the Global Atopic Dermatitis Atlas (GADA)

Dermatology-oncolog