International bullous diseases group: consensus on diagnostic criteria for epidermolysis bullosa acquisita

BACKGROUND: Epidermolysis bullosa acquisita (EBA) is a complex autoimmune bullous disease disease with variable clinical presentations and multiple possible diagnostic tests, making an international consensus on the diagnosis of EBA essential. ----- OBJECTIVES: To obtain an international consensus on the clinical and diagnostic criteria for EBA. ----- METHODS: The International Bullous Diseases Group (IBDG) met three times to discuss the clinical and diagnostic criteria for EBA. For the final voting exercise, 22 experts from 14 different countries voted on 50 different items. When > 30% disagreed with a proposal, a discussion was held and re-voting carried out. ----- RESULTS: In total, 48 of 50 proposals achieved consensus after discussion. This included nine diagnostic criteria, which are summarized in a flow chart. The IBDG was unable to determine one procedure that would be applicable worldwide. A limitation of the study is that differential diagnosis of bullous systemic lupus erythematosus has not been addressed. ----- CONCLUSIONS: This first international consensus conference established generally agreed-upon clinical and laboratory criteria defining the clinical classification of and diagnostic testing for EBA. Holding these voting exercises in person with the possibility of discussion prior to voting has advantages in reaching consensus over Delphi exercises with remote voting

Report from the fourth international consensus meeting to harmonize core outcome measures for atopic eczema/dermatitis clinical trials (HOME initiative)

This article is a report of the fourth meeting of the Harmonising Outcome Measures for Eczema (HOME) initiative held in Malmö, Sweden on 23–24 April 2015 (HOME IV). The aim of the meeting was to achieve consensus over the preferred outcome instruments for measuring patient-reported symptoms and quality of life for the HOME core outcome set for atopic eczema (AE). Following presentations, which included data from systematic reviews, consensus discussions were held in a mixture of whole group and small group discussions. Small groups were allocated a priori to ensure representation of different stakeholders and countries. Decisions were voted on using electronic keypads. For the patient-reported symptoms, the group agreed by vote that itch, sleep loss, dryness, redness/inflamed skin and irritated skin were all considered essential aspects of AE symptoms. Many instruments for capturing patient-reported symptoms were discussed [including the Patient-Oriented SCOring Atopic Dermatitis index, Patient-Oriented Eczema Measure (POEM), Self-Administered Eczema Area and Severity Index, Itch Severity Scale, Atopic Dermatitis Quickscore and the Nottingham Eczema Severity Score] and, by consensus, POEM was selected as the preferred instrument to measure patient-reported symptoms. Further work is needed to determine the reliability and measurement error of POEM. Further work is also required to establish the importance of pain/soreness and the importance of collecting information regarding the intensity of symptoms in addition to their frequency. Much of the discussion on quality of life concerned the Dermatology Life Quality Index and Quality of Life Index for Atopic Dermatitis; however, consensus on a preferred instrument for measuring this domain could not be reached. In summary, POEM is recommended as the HOME core outcome instrument for measuring AE symptoms

238U and 230Th Excesses in Kolbeinsey Ridge Basalts

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International collaboration

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Understanding melt generation beneath the slow-spreading Kolbeinsey Ridge using ²³⁸U, ²³⁰Th, and ²³¹Pa excesses

To examine the petrogenesis and sources of basalts from the Kolbeinsey Ridge, one of the shallowest locations along the global ridge system, we present new measurements of Nd, Sr, Hf, and Pb isotopes and U-series disequilibria on 32 axial basalts. Young Kolbeinsey basalts (full-spreading rate = 1.8 cm/yr; 67°05'–70°26'N) display (230Th/238U) 230Th/238U) > 1 with (230Th/238U) from 0.95 to 1.30 and have low U (11.3–65.6 ppb) and Th (33.0 ppb–2.40 ppm) concentrations. Except for characteristic isotopic enrichment near the Jan Mayen region, the otherwise depleted Kolbeinsey basalts (e.g. 87Sr/86Sr = 0.70272–0.70301, eNd = 8.4–10.5, eHf = 15.4–19.6 (La/Yb)N = 0.28–0.84) encompass a narrow range of (230Th/232Th) (1.20–1.32) over a large range in (238U/232Th) (0.94–1.32), producing a horizontal array on a (230Th/232Th) vs. (238U/232Th) diagram and a large variation in (230Th/238U). However, the (230Th/238U) of the Kolbeinsey Ridge basalts (0.96–1.30) are inversely correlated with (234U/238U) (1.001–1.031). Samples with low (230Th/238U) and elevated (234U/238U) reflect alteration by seawater or seawater-derived materials. The unaltered Kolbeinsey lavas with equilibrium 234U/238U have high (230Th/238U) values (?1.2), which are consistent with melting in the presence of garnet. This is in keeping with the thick crust and anomalously shallow axial depth for the Kolbeinsey Ridge, which is thought to be the product of large degrees of melting in a long melt column. A time-dependent, dynamic melting scenario involving a long, slowly upwelling melting column that initiates well within the garnet peridotite stability zone can, in general, reproduce the (230Th/238U) and (231Pa/235U) ratios in uncontaminated Kolbeinsey lavas, but low (231Pa/235U) ratios in Eggvin Bank samples suggest eclogite involvement in the source for that ridge segment

Understanding melt generation beneath the slow-spreading Kolbeinsey Ridge using U-238, Th-230, and Pa-231 excesses

To examine the petrogenesis and sources of basalts from the Kolbeinsey Ridge, one of the shallowest locations along the global ridge system, we present new measurements of Nd, Sr, Hf, and Pb isotopes and U-series disequilibria on 32 axial basalts. Young Kolbeinsey basalts (full-spreading rate=1.8cm/yr; 67°05'-70°26'N) display (230Th/238U)1 with (230Th/238U) from 0.95 to 1.30 and have low U (11.3-65.6ppb) and Th (33.0ppb-2.40ppm) concentrations. Except for characteristic isotopic enrichment near the Jan Mayen region, the otherwise depleted Kolbeinsey basalts (e.g. 87Sr/86Sr=0.70272-0.70301, εNd=8.4-10.5, εHf=15.4-19.6 (La/Yb)N=0.28-0.84) encompass a narrow range of (230Th/232Th) (1.20-1.32) over a large range in (238U/232Th) (0.94-1.32), producing a horizontal array on a (230Th/232Th) vs. (238U/232Th) diagram and a large variation in (230Th/238U). However, the (230Th/238U) of the Kolbeinsey Ridge basalts (0.96-1.30) are inversely correlated with (234U/238U) (1.001-1.031). Samples with low (230Th/238U) and elevated (234U/238U) reflect alteration by seawater or seawater-derived materials. The unaltered Kolbeinsey lavas with equilibrium 234U/238U have high (230Th/238U) values (≥1.2), which are consistent with melting in the presence of garnet. This is in keeping with the thick crust and anomalously shallow axial depth for the Kolbeinsey Ridge, which is thought to be the product of large degrees of melting in a long melt column. A time-dependent, dynamic melting scenario involving a long, slowly upwelling melting column that initiates well within the garnet peridotite stability zone can, in general, reproduce the (230Th/238U) and (231Pa/235U) ratios in uncontaminated Kolbeinsey lavas, but low (231Pa/235U) ratios in Eggvin Bank samples suggest eclogite involvement in the source for that ridge segment. © 2011 Elsevier Ltd.F.I. 4, 101SCOPUS: ar.jinfo:eu-repo/semantics/publishe

Development of a quality-of-life instrument for autoimmune bullous disease: The autoimmune bullous disease quality of life questionnaire

IMPORTANCE Quality-of-life (QOL) evaluation is an increasingly important outcome measure in dermatology, with disease-specific QOL instruments being the most sensitive to changes in disease status. OBJECTIVE To develop a QOL instrument specific to autoimmune bullous disease (AIBD). DESIGN A comprehensive item generation process was used to build a 45-item pilot Autoimmune Bullous Disease Quality of Life (ABQOL) questionnaire, distributed to 70 patients with AIBD. Experts in bullous disease refined the pilot ABQOL before factor analysis was performed to yield the final ABQOL questionnaire of 17 questions.We evaluated validity and reliability across a range of indices. SETTING Australian dermatology outpatient clinics and private dermatology practices. PATIENTS AND EXPOSURE Patients with a histological diagnosis of AIBD. MAIN OUTCOMES AND MEASURES The development of an AIBD-specific QOL instrument. RESULTS Face and content validity were established through the comprehensive patient interview process and expert review. In terms of convergent validity, the ABQOL was found to have a moderate correlation with scores on the Dermatology Life Quality Index (R = 0.63) and the General Health subscale of the 36-Item Short Form Health Survey (R = 0.69; P = .009) and low correlation with the Pemphigus Disease Area Index (R = 0.42) and Autoimmune Bullous Disease Skin Disorder Intensity Score (R = 0.48). In terms of discriminant validity, the ABQOL was found to be more sensitive than the Dermatology Life Quality Index (P = .02). The ABQOL was also found to be a reliable instrument evaluated by internal consistency (Cronbach α coefficient, 0.84) and test-retest reliability (mean percentage variation, 0.92). CONCLUSIONS AND RELEVANCE The ABQOL has been shown to be a valid and reliable instrument that may serve as an end point in clinical trials. Future work should include incorporating patient weighting on questions to further increase content validity and translation of the measure to other languages