Quality of abstracts in 3 clinical dermatology journals

Background Structured abstracts have been widely adopted in medical journals, with little demonstration of their superiority over unstructured abstracts.Objectives: To compare abstract quality among 3 clinical dermatology journals and to compare the quality of structured and unstructured abstracts within those journals. Design and Data Sources: Abstracts of a random sample of clinical studies (case reports, case series, and reviews excluded) published in 2000 in the Archives of Dermatology, The British Journal of Dermatology, and the Journal of the American Academy of Dermatology were evaluated. Each abstract was rated by 2 independent investigators, using a 30-item quality scale divided into 8 categories (objective, design, setting, subjects, intervention, measurement of variables, results, and conclusions). Items applicable to the study and present in the main text of the article were rated as being present or absent from the abstract. A global quality score (range, 0-1) for each abstract was established by calculating the proportion of criteria among the eligible criteria that was rated as being present. A score was also calculated for each category. Interrater agreement was assessed with a {kappa} statistic. Mean ± SD scores were compared among journals and between formats (structured vs unstructured) using analysis of variance. Main Outcome Measures: Mean quality scores of abstracts by journal and by format. Results: Interrater agreement was good ({kappa} = 0.71). Mean ± SD quality scores of abstracts were significantly different among journals (Archives of Dermatology, 0.78 ± 0.07; The British Journal of Dermatology, 0.67 ± 0.17; and Journal of the American Academy of Dermatology, 0.64 ± 0.15; P = .045) and between formats (structured, 0.71 ± 0.11; and unstructured, 0.56 ± 0.18; P = .002). The setting category had the lowest scores. Conclusions: The quality of abstracts differed across the 3 tested journals. Unstructured abstracts were demonstrated to be of lower quality compared with structured abstracts and may account for the differences in quality scores among the journals. The structured format should be more widely adopted in dermatology journals

Efficacité des immunoglobulines intraveineuses dans les formes cutanées pures de dermatomyosite

Le traitement des formes cutanées pures de dermatomyosite (DM) n est pas codifié. Le traitement par immunoglobulines intraveineuses (IgIV) est recommandé dans les DM corticorésistantes ou corticodépendantes mais seuls 8 cas isolés d utilisation des IgIV dans l atteinte cutanée pure de DM ont été rapportés. L objectif est d évaluer l efficacité et la tolérance des IgIV dans l atteinte cutanée de DM sans atteinte musculaire. Nous avons mené une étude rétrospective de 27 patients traités par IgIV pour une atteinte cutanée sévère de DM (sans atteinte musculaire ou atteinte musculaire non symptomatique) après échec de la photoprotection et au moins une ligne de traitement. Dix-neuf patients présentaient une réponse complète ou quasi-complète, 4 patients présentaient une réponse partielle et 4 patients ne présentaient pas de réponse, incluant 2 patients ayant présenté des effets secondaires de grade 3 (céphalées). Le nombre moyen de cures était de 4,8 (1 à 15). Dix patients (53%) présentaient une rechute, avec un délai moyen de 6,2 mois après la dernière cure d IgIV. Six patients étaient traités avec succès par de nouvelles cures d IgIV. Une progression musculaire de la DM était observée chez 6 patients. Les IgIV semblent efficaces et bien tolérées dans le traitement des formes cutanées isolées de DM. Les rechutes sont fréquentes mais sont accessibles à de nouvelles cures d IgIV. Une étude contrôlée serait nécessaire pour confirmer ces résultats.PARIS6-Bibl.Pitié-Salpêtrie (751132101) / SudocSudocFranceF

Early necrotizing fasciitis following initiation of mycophenolate mofetil in two patients with bullous pemphigoid

International audienceno abstrac

Clinical Spectrum, Quality of Life, BRAF Mutation Status and Treatment of Skin Involvement in Adult Langerhans Cell Histiocytosis

Langerhans cell histiocytosis is a rare histiocytic disorder for which skin involvement and management are poorly described in adults. The aim of this retrospective monocentric study in a national reference centre is to describe the clinical characteristics, quality of life, BRAF mutation status and outcomes of skin involvement in adult patients with Langerhans cell histiocytosis. Twenty-five patients (14 females, mean age 47 years) were included, with a median follow-up of 33 months (range 4–420 months). Patients experienced poor dermatological quality of life despite low body surface involvement. BRAFV600 mutations were detected in 8 of the 18 patients analysed (45%). Eight patients had an associated malignancy. Several treatment options were used and consisted of surgery, topical steroids and carmustine, thalidomide, methotrexate, vinblastine and steroids and cladribine. This study highlights the need to evaluate quality of life and to screen for associated malignancy in adult patients with Langerhans cell histiocytosis

Active chronic sarcoidosis is characterized by increased transitional blood B cells, increased IL-10-producing regulatory B cells and high BAFF levels.

BACKGROUND: Sarcoidosis is a multisystemic disease of unknown etiology characterized by a disproportionate Th1 granulomatous immune response in the organs involved. Plasmatic hypergammaglobulinemia and B cell accumulation in granulomatous lesions suggest the possible role of humoral immune responses in the pathogenesis of sarcoidosis. The purpose of this study is to describe B cell peripheral compartment in sarcoidosis. METHODOLOGY/PRINCIPAL FINDINGS: We analyzed blood B cell subsets and BAFF levels in 33 patients with chronic sarcoidosis (active sarcoidosis n = 18; inactive sarcoidosis n = 15) and 18 healthy donors. Active chronic sarcoidosis patients had significantly less circulating memory B cells (p<0.01), more transitional (p<0.01) and increased numbers of IL-10-producing regulatory B cells (p<0.05) compared with healthy donors and patients with inactive sarcoidosis. BAFF serum levels were significantly higher in patients with active sarcoidosis (p<0.01 versus healthy donors and inactive sarcoidosis patients) and strongly correlated with serum hypergammaglobulinemia (r = 0.53, p<0.01) and angiotensin converting enzyme levels (r = 0.61, p = <0.01). CONCLUSIONS/SIGNIFICANCE: These data show that there is an altered B cell homeostasis in active sarcoidosis and suggest BAFF antagonist drugs as potential new treatments of this disease

An ACT1 Mutation Selectively Abolishes Interleukin-17 Responses in Humans with Chronic Mucocutaneous Candidiasis

International audiencePatients with inborn errors of interleukin-17F (IL-17F) or IL-17RA display chronic mucocutaneous candidiasis (CMC). We report a biallelic missense mutation (T536I) in the adaptor molecule ACT1 in two siblings with CMC. The mutation, located in the SEFIR domain, abolished the homotypic interaction of ACT1 with IL-17 receptors, with no effect on homodimerization. The patients' fibroblasts failed to respond to IL-17A and IL-17F, and their T cells to IL-17E. By contrast, healthy individuals homozygous for the common variant D10N, located in the ACT1 tumor necrosis factor receptor-associated factor-interacting domain and previously associated with psoriasis, had impaired, but not abolished, responses to IL-17 cytokines. SEFIR-independent interactions of ACT1 with other proteins, such as CD40, heat shock protein 70 (HSP70) and HSP90, were not affected by the T536I mutation. Overall, human IL-17A and IL-17F depend on ACT1 to mediate protective mucocutaneous immunity. Moreover, other ACT1-dependent IL-17 cytokines seem to be largely redundant in host defense