A histopathological classification system of Tyr::NRASQ61Kmurine melanocytic lesions: A reproducible simplified classification

Abstract

Genetically engineered mouse models offer essential opportunities to investigate the mechanisms of initiation and progression in melanoma. Here we report a new simplified histopathology classification of mouse melanocytic lesions in Tyr::NRAS^Q61K derived models, using an interactive decision tree that produces homogeneous categories. Reproducibility for this classification system was evaluated on a panel of representative cases of murine melanocytic lesions by pathologists and basic scientists. Reproducibility, measured as inter-rater agreement between evaluators using a modified Fleiss' kappa statistic revealed a very good agreement between observers. Should this new simplified classification be adopted, it would create a robust system of communication between researchers in the field of mouse melanoma models.

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Fenómeno de Lucio. Comunicación de 14 casos

Publication date: Available online 9 December 2017
Source:Piel
Author(s): Arnaldo Aldama, Víctor Wattiez, Gloria Mendoza
IntroducciónEl fenómeno de Lucio es una leprorreacción grave, observada principalmente en la lepra lepromatosa difusa, caracterizada por lesiones necróticas por vasculitis.Material y métodoEstudio retrospectivo de los casos observados en el servicio de Dermatología del Hospital Nacional de Paraguay en el período 1990-2016. Se incluyen pacientes de todas las edades, de ambos sexos, con confirmación histológica.ResultadosEn el período mencionado se diagnosticaron 14 casos de fenómeno de Lucio, 9 del sexo masculino, con edades que oscilaban entre 35 y 87 años (promedio de 61,9 años, DE: ±14,54). Todos fueron ingresados en el hospital, 13 por el servicio de Urgencias y uno por el de Dermatología. Solo un paciente se conocía previamente enfermo de lepra; en los demás, la reacción posibilitó el diagnóstico de la enfermedad. La mayoría fue la forma lepromatosa de la variedad difusa, pero 5 tenían lesiones tuberosas y en uno el informe histológico fue de borderline lepromatosa. Tres pacientes fallecieron durante el ingreso y a 3 se les practicó amputación de miembros, todos por infecciones piógenas sobreagregadas. De los 11 pacientes dados de alta hospitalaria, 9 completaron adecuadamente el tratamiento y fueron dados de alta terapéutica. El manejo consistió en medidas de soporte, tratamiento de las complicaciones y las intercurrencias, y en poliquimioterapia específica para la lepra multibacilar. Ocho pacientes recibieron prednisona.ConclusiónEl fenómeno de Lucio es un cuadro grave con elevada morbimortalidad, que debe ser conocido no solo por el dermatólogo sino por médicos de otras especialidades.IntroductionThe Lucius Phenomenon is a severe reaction, mainly observed in diffuse lepromatous leprosy, and characterised by necrotic lesions due to vasculitis.Material and methodRetrospective study of the cases observed in the Dermatology Department of the National Hospital of Paraguay in the period 1990-2016. The study included patients of all ages, and both sexes with histological confirmation.ResultsIn the mentioned period, a total of 14 cases of Lucio's phenomenon were diagnosed during the study period, of whom 9 were male, with ages ranging from 35 to 87 years (mean 61.9 years, SD: ±14.54). All were admitted to the Hospital, 13 by the Emergency Department, and one by Dermatology. Only one patient was known to be suffering from leprosy previously, whereas in others the reaction presented led to the diagnosis of the disease. Most were lepromatous leprosy of the diffuse variety, but 5 had tuberous lesions, and in one the histological report was lepromatous borderline. Three patients died during admission, and three suffered amputation of all limbs due to overgenerated pyogenic infections. Of the 11 patients discharged, 9 successfully completed the treatment and were given high-dose therapy. Management consisted of supportive measures, treatment of complications and recurrences, multiple drug therapy specific for multibacillary leprosy. Eight patients received prednisone.ConclusionLucio's phenomenon is a serious clinical condition with a high morbidity and mortality, which must be known not only by the dermatologist but by doctors of other specialties.

Is Neutrophilic Desquamative Erythroderma a Form of Acute Generalized Exanthematous Pustulosis?

Abstract

Delayed-type hypersensitivity to systemically administered corticosteroids is rare. Corticosteroids are unique allergens, potentially leading to atypical manifestations. They are commonly used for treatment of allergic, inflammatory and autoimmune diseases because of intrinsically immunosuppressive and immune-modulatory properties (1). These properties may counterbalance, mask and delay allergic reactions as has been shown (1). It has been speculated that allergic reactions to corticosteroids may remain unrecognized due to their broad clinical variability or frequently atypical presentation (1).

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Facial Swelling and Foreign Body Granulomatous Reaction to Hyaluronic Acid Filler in the setting of Neratinib

Abstract

Cosmetic injection of dermal fillers is common, with late complications increasingly recognized. Herein, we report a granulomatous reaction to hyaluronic acid filler occurring during the use of neratinib. A woman in her fifties, with metastatic adenocarcinoma of unknown primary involving the skin and bones, was referred for new-onset facial swelling. Notable past medical history included progression of disease on carboplatin/paclitaxel and a hemithyroidectomy for papillary thyroid cancer.

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Ultrastructural Aspects of Hairs of Chediak-Higashi Syndrome

Abstract

Chediak-Higashi syndrome (CHS) belongs to a group of partial oculocutaneous albinism and immunodeficiency. Five autosomal recessive conditions are recognized in this group, characterized by hypopigmentation of hair, skin and eyes, associated with recurrent infections. Additionally CHS can present coagulopathies, neurological dysfunction and large granules in many cell types.

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Is there a pathogenetic link between frontal fibrosing alopecia, androgenetic alopecia, and fibrosing alopecia in a pattern distribution?

Abstract

Frequent coexistence of frontal fibrosing alopecia (FFA) and androgenetic alopecia (AGA) has been reported in the literature, but little attention has been given to the pathogenetic mechanisms underlying this potential association. FFA is characterized by progressive cicatricial fronto-temporal hairline recession, mostly affecting peri-menopausal women. Immune mechanisms seem to play a pivotal pathogenetic role.

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Bizarre appearance of chronic cutaneous lupus erythematosus of face mimicking factitial dermatitis

Abstract

Old dermatological wisdom says that syphilis and lupus are great imitators. Cutaneous manifestations of lupus erythematosus (LE) are usually characteristic, however, some patients may present with a less typical clinical picture. Correct diagnosis of cutaneous lupus erythematosus (CLE) requires a high level of clinical suspicion and understanding of broad differential diagnosis. Early lesions of CLE are sometimes difficult to diagnose, both clinically and histopathologically.

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Phototherapy in France: quantitative data (2007-2016) from the National Health Insurance register

Abstract

Phototherapy is a cost-effective treatment with a safe side-effect profile for the management of a wide range of dermatoses. Although one of the most common indications for phototherapy is psoriasis, the use of phototherapy for psoriasis has seen a decline in the U.S.A. Data from the National Ambulatory Medical Care Survey indicate a 94% decrease of physician visits for phototherapy between 1994 and 1998.

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Dermoscopic observations in disseminated cryptococcosis with cutaneous involvement

Abstract

A 26-year-old female patient with a history of intravenous drug abuse, diagnosed with acquired immunodeficiency syndrome (AIDS) 6 months before. AIDS defining disease was Pneumocystis jiroveci pneumonia. The patient presented with severely decreased CD4 cell count of 7 cells/mm³ and relatively low HIV viral load of 281 copies/mL. Shortly after initiation of antiretroviral therapy (ART), was diagnosed with disseminated cryptococcosis with central nervous system and skin involvement.

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Power Doppler ultrasound assessment of vascularization in hidradenitis suppurativa lesions

Abstract

Background

Ultrasound and Power Doppler (PD) ultrasound are useful tools to study and monitor Hidradenitis Suppurativa (HS) patients.

Objective

Describe the Power Doppler signal of HS nodules, abscesses, fistulas.

Methods

A retrospective analysis of PD in mild, moderate, severe HS patients, collecting all demographic and clinical data. The lesions were classified according to their ultrasound morphology, describing the vascular degree - high, moderate, minimal - and distribution - peripheral, internal, mixed. Statistical analysis was performed using odds ratio and bivariate regression.

Results

A total of 241 lesions, 62 nodules, 64 abscesses, 99 simple fistulas, 16 complex fistulas, from 61 HS patients, were included. Vascular distribution was defined peripheral in 143/241, mixed in 55/241, internal in 0/241 lesions, regardless the clinical type. Qualitative Doppler showed high vascularization in 44/241 lesions, moderate in 79/241, minimal in 75/241, despite the clinical type. All lesions showed resistive index <0.7. Age, disease's duration, size of the lesions, high Sartorius score, high BMI, showed positive statistically correlation with both PD signal and mixed vascular distribution. No statistically significance was evidenced for vascular degree measurements.

Limitations

Ultrasound cannot detect lesions <0.1 mm.

Conclusion

Vascular distribution of HS lesions can be evaluated by PD with additional relevant information for earlier and better disease management.

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Progressive osseous heteroplasia in a Chinese infant and a novel mutation in the GNAS Gene

Abstract

Progressive osseous heteroplasia(POH,OMIM 166350) is a rare inherited disease that begins with skin ossification and proceeds into the deeper connective tissues. Most cases of POH are caused by heterozygous inactivating mutations of GNAS gene. Herein, we report a new sporadic case of atypical POH and its underlying genetic basis.

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Pili annulati in a case of Rothmund-Thomson syndrome with a novel frameshift mutation in RECQL4

Abstract

Rothmund-Thomson syndrome (RTS) is a rare autosomal recessive genodermatosis characterised by erythematous patches or plaques and sometimes swelling and blisters, which appear during infancy on photo-exposed sites, usually the cheeks, then progress to poikiloderma. Other features include gastrointestinal disturbance, short stature, sparse scalp hair/eyebrows/eyelashes, skeletal abnormalities, juvenile cataracts, hypogonadism and a susceptibility to malignancy. Two forms have been described: Type I, characterised by poikiloderma, ectodermal dysplasia and juvenile cataracts, negative for the RECQL4 mutation, and Type II, with poikiloderma, congenital bone defects and increased risk of osteosarcoma related to deleterious RECQL4 mutations.

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Leiomiosarcoma cutáneo: la importancia de la localización histológica

Publication date: Available online 9 December 2017
Source:Actas Dermo-Sifiliográficas
Author(s): B. Llombart

Photodynamic therapy in the treatment of symptomatic oral lichen planus: A systematic review

Abstract

Background

The aim of this systematic review was to assess the efficacy of photodynamic therapy (PDT) in the treatment of symptomatic oral lichen planus (OLP).

Methods

This systematic review aimed to address the following focused question: "Is PDT effective in the treatment of symptomatic OLP?" Indexed databases; MEDLINE, EMBASE and CENTRAL were searched up to and including August 2017.

Results

Six clinical studies were included. The risk of bias was considered high in five studies and moderate in one study. Parameters of photodynamic therapy such as wavelengths, energy fluence, power density and exposure time ranged between 320-660 nm, 120 J cm⁻², 130 mW cm⁻², and 70-150 s, respectively. The follow up period ranged from 4 to 48 weeks. All included studies reporting clinical scores showed that PDT was effective in the treatment of OLP in adult patients at follow-up. However, PDT did not showed significant improvement when compared with steroid therapy.

Conclusion

PDT appears to have some effect in the symptomatic treatment of OLP in adult patients. However, further randomized controlled trials with long follow-up period, standardized PDT parameters and comparing efficacy of PDT with steroid therapy are warranted to obtain strong conclusions in this regard.

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The Prevalence and Odds of Depression in Patients with Vitiligo: a Meta-Analysis

Abstract

Objective

The objective of this study is to provide a pooled estimate of the prevalence and odds of depression in Vitiligo patients.

Methods

A systematic literature search of Medline, Embase, and Cochrane library databases was conducted. The proportions and mean questionnaire values for depression were pooled according to different evaluation methods. In controlled studies, odds ratio (OR) and standardized mean differences (SMDs) compared depression in vitiligo patients with healthy controls.

Results

One thousand nine hundred and sixty-five patients were identified from 20 eligible cohorts to be included in the present meta-analysis review. Sample sizes ranged from 30 to 308 for each analysis. The pooled prevalence of depression across 17 unique populations (n = 1,711) was 29% (95% CI 20 - 39). The prevalence of clinical depression was 8% (95%CI 2-14%) using Diagnostic and Statistical Manual of Mental Disorders IV (DSM IV) or International Classification of Diseases codes-10 (ICD 10 and the prevalence of depressive symptoms was 33% (95%CI: 23-44%) using validated screening inventory and rating scales. Vitiligo patient were 4.96 times more likely to display depression compared with controls. Subgroup analysis showed that the prevalence of depression in Asian and female vitiligo patients were significantly higher than those in Caucasian and male ones. The pooled prevalence of depressive symptoms was higher (56%, 95%CI: 0.5-0.62) and the heterogeneity was lowering cases with Hamilton Depression Rating Scale (HDRS) questionnaire when compared with other questionnaires.

Conclusions

This meta-analysis review indicates that the prevalence of depression is high in vitiligo patients. The HDRS questionnaire may be the most sensitive tool for testing the depressive symptoms among vitiligo patients.

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Children with atopic dermatitis may have unacknowledged contact allergies contributing to their skin symptoms

Abstract

Background

Whether children with atopic dermatitis have an altered risk of contact allergy than children without atopic dermatitis is frequently debated and studies have been conflicting. Theoretically, the impaired skin barrier in AD facilitates the penetration of potential allergens and several authors have highlighted the risk of underestimating and overlooking contact allergy in children with atopic dermatitis.

Objective

To determine the prevalence of contact allergy in Danish children with atopic dermatitis and explore the problem of unacknowledged allergies maintaining or aggravating the skin symptoms.

Methods

In a cross sectional study, 100 children and adolescents aged 5-17 years with a diagnosis of atopic dermatitis were patch tested with a pediatric series of 31 allergens.

Results

Thirty percent of the children had at least one positive patch test reaction and 17% had at least one contact allergy that was relevant to the current skin symptoms. The risk of contact allergy was significantly correlated to the severity of atopic dermatitis. Metals and components of topical skin care products were the most frequent sensitizers.

Conclusion

Patch testing is relevant as a screening tool in the management of children with atopic dermatitis as they may have unacknowledged contact allergies contributing to or maintaining their skin symptoms. Children with atopic dermatitis seem to be at greater risk of sensitization to certain allergens including metals and components of skin care products.

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Safety and Efficacy of guselkumab in Japanese patients with moderate-to-severe plaque psoriasis: A Randomised, Placebo-Controlled, Ascending Dose Study

Summary

Background

The Interlukin-23 (IL-23)/Interlukin-17 (IL-17) pathway is central in the pathogenesis of psoriasis. The favourable efficacy and safety of guselkumab, an IL-23 specific monoclonal antibody, has been demonstrated in global Phase 3 studies of plaque psoriasis.

Objectives

To evaluate the safety, efficacy and pharmacokinetics of a single-dose subcutaneous guselkumab in Japanese patients with moderate-to-severe plaque psoriasis.

Methods

Patients with ≥ 10% of total body surface area (BSA) involvement and a Psoriasis Area and Severity Index (PASI) ≥12 were randomised (5:1) to receive guselkumab or placebo in 4 cohorts of this double-blind, placebo-controlled, single ascending dose, single-centre study. Safety, pharmacokinetics, and clinical response were monitored at baseline and specific time points over a 24-wk follow-up period.

Results

Through week 24, 54% (11/20) of guselkumab patients and 50% (2/4) of placebo patients experienced ≥1 adverse event (AE). No deaths, serious AEs, or AEs leading to treatment discontinuation were reported. Maximum clinical response was seen at week 16 with PASI 75 response in 2/5 (10 mg), 4/5 (30 mg and 300 mg), and 3/5 (100 mg) patients and PASI 90 in 0/5 (10 mg), 3/5 (30 mg), 2 /5 (100 mg) and 3/5 (300 mg) patients. Mean C_max and AUC_∞ values increased in a dose-proportional manner with mean terminal t_1/2 of 15.6-17.6 days and median t_max of 4-6 days.

Conclusions

Guselkumab was generally well-tolerated and exhibited sustained high levels of clinical response in Japanese patients with moderate-to-severe psoriasis.

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Which outcomes are reported in cellulitis trials? Results of a review of outcomes included in cellulitis trials and a patient priority setting survey

Summary

Background

There is an emerging need to develop consistent outcomes in clinical trials to allow effective comparison of treatment effects. No systematic review has previously looked at the reporting of outcome measures used in randomised controlled trials (RCTs) on treatment and prevention of cellulitis (erysipelas).

Objectives

The primary aim of this review was to describe the breadth of outcomes reported from RCTs on cellulitis treatment and prevention. The secondary aim was to identify outcome themes from patient and health care professionals' feedback from a cellulitis priority setting partnership (PSP).

Methods

We conducted a review of all outcome measures used in RCTs from two recent Cochrane reviews. Free text responses from a cellulitis priority setting survey were used to understand the perspectives of patients and healthcare professionals.

Results

Outcomes from 42 RCTs on treatment of cellulitis and six RCTs on prevention of cellulitis were reviewed. Only 28 trials stated their primary outcome. For trials assessing treatment of cellulitis, clinical response to treatment was categorised in 25 different ways. Five of these trials used an outcome that was in accordance with FDA guidance and only four trials incorporated either quality of life or patient satisfaction. For trials assessing prevention of cellulitis, recurrence was the key outcome measure. From the cellulitis PSP, prevention of recurrence, clinical features and long-term disease impact were the most important outcome themes for patients.

Conclusions

We have shown that in cellulitis treatment and prevention research, there is significant heterogeneity in clinical outcomes, inadequate focus on patient-reported outcomes, and a disparity between what is currently measured and what patients and healthcare professionals feel is important. We recommend that future cellulitis treatment trials consider the use of longer-term outcomes to capture recurrence and long-term morbidity, as well as short-term resolution of acute infection.

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Evaluation of the anti-inflammatory and urotoxicity ameliorative effects of γ-humulene containing active fraction of Emilia sonchifolia (L.) DC.

Abstract

In folklore medicine, the plant Emilia sonchifolia, belonging to the family Asteraceae, is used for treating tumour and inflammation. In our previous studies, we have done a thorough phytochemical investigation of E. sonchifolia with a report on its potent antimetastatic activity. Further, we isolated and characterised its active fraction (AFES) containing the major compound γ-humulene with an evaluation of the antiangiogenic effect of AFES (5 mg/kg b.wt.). In the first part of the present study, AFES in different concentrations was used for the assessment of its possible anti-inflammatory effect employing three in vivo inflammatory models. Further using the most effective concentration of AFES 5 mg/kg b.wt, its effect on proinflammatory cytokine levels was recorded along with a confirmatory gene expression analysis. The results manifested with a reduction in the paw oedema significantly decreased levels of proinflammatory cytokines, C-reactive protein, nitric oxide and also there was an efficient downregulation of cyclooxygenase-2 and inducible nitric oxide. Urotoxicity is one of the major side effects of conventional chemotherapy. So in the second part of the study, we used AFES in combination with the conventional therapeutic agent cyclophosphamide in vivo in mice. The effect of AFES on urotoxicity was assessed from various biochemical parameters, cytokine markers and finally with a histopathology of the bladder. The current study revealed the protective effects of AFES, implicating reduced levels of urea nitrogen, by revamping of glutathione and marker cytokine levels towards positive amelioration. The results obtained altogether proved the safeguarding effect of AFES in murine experimental models.

Comparison of Elliptical Excision versus Punch Incision for the Treatment of Epidermal Inclusion Cysts: A Prospective, Randomized Study

Publication date: Available online 9 December 2017
Source:Journal of the American Academy of Dermatology
Author(s): Justin Cheeley, Laura Delong Aspey, Jamie MacKelfresh, Michelle Pennie, Suephy Chen

Merkel Cell Carcinoma: an Update and Review Part 1. Pathogenesis, Diagnosis, and Staging

Publication date: Available online 9 December 2017
Source:Journal of the American Academy of Dermatology
Author(s): Kathleen Coggshall, Tiffany L. Tello, Jeffrey P. North, Siegrid S. Yu
Merkel cell carcinoma (MCC) is an uncommon primary cutaneous neuroendocrine cancer. It most commonly presents as an indurated plaque or nodule on sun-damaged skin in elderly patients and is characterized by high rates of local recurrence and nodal metastasis. Survival at five years is 51% for local disease and as low as 14% for distant disease, which underscores the aggressive nature of this tumor and challenges in management. Advances in immunology and molecular genetics have broadened our understanding of the pathophysiology of MCC and expanded our therapeutic arsenal. With this comprehensive review, we provide an update of MCC epidemiology, pathogenesis, clinical presentation, diagnostic evaluation and prognostic markers. Part II of this review will explore the evolving landscape in MCC management.

Teaser

Capsule Summary: word count not required for CME articles; please note that bulleted points appear at the start of each section but are not included in the total word count

Anti-melanoma differentiation-associated gene 5 (MDA5) dermatomyositis: a concise review with an emphasis on distinctive clinical features

Publication date: Available online 9 December 2017
Source:Journal of the American Academy of Dermatology
Author(s): Drew JB. Kurtzman, Ruth Ann Vleugels
Melanoma differentiation-associated gene 5 (MDA5) is a recently described autoantigen target in a subset of patients with dermatomyositis (DM). Anti-MDA5 DM is characterized by a unique mucocutaneous and systemic phenotype that includes cutaneous and oral ulceration, painful palmar papules, alopecia, panniculitis, arthritis, a lower incidence of myositis, and, importantly, an elevated risk of interstitial lung disease, with a potentially fatal course. Because the clinical features may differ substantially from those typically observed in cutaneous DM, the diagnosis is often overlooked, which may negatively affect patient outcomes. This review aims to familiarize the clinician with the distinctive clinical features of anti-MDA5 DM in order to enhance its recognition and to facilitate an appropriate screening and management strategy.

Teaser

Anti-MDA5 dermatomyositis exhibits unique clinical features including mucocutaneous ulceration, palmar papules, non-scarring alopecia, panniculitis, arthritis, and interstitial lung disease.Early intervention may reduce disease-related morbidity and improve survival in affected individuals.Knowledge of the salient features of anti-MDA5 dermatomyositis enhances its recognition and facilitates treatment.

‘‘Intralesional Drainage Injection’ of Triamcinolone acetonide for Epidermal Cyst’

Publication date: Available online 9 December 2017
Source:Journal of the American Academy of Dermatology
Author(s): Deepak Jakhar, Ishmeet Kaur

Merkel Cell Carcinoma: An Update and Review. Part II: Current and Future Therapy

Publication date: Available online 9 December 2017
Source:Journal of the American Academy of Dermatology
Author(s): Tiffany L. Tello, Kathleen Coggshall, Sue S. Yom, Siegrid S. Yu
Merkel cell carcinoma (MCC) is a rare neuroendocrine tumor of the skin associated with a high risk of local recurrence and distant metastases. It most commonly occurs on sun-exposed areas of Caucasian patients over the age of 65.^1,2 The Merkel cell polyomavirus (MCV) is thought to be responsible for malignant transformation in approximately 80% of cases in the northern hemisphere,³ while ultraviolet radiation induced DNA damage is implicated in MCV-negative tumors.⁴ The overall incidence of MCC is low, with approximately 1600 cases diagnosed annually in the United States. The rate is much higher in patients with lymphoproliferative malignancies, solid organ transplants, and HIV infection.^5–9 The low overall incidence of this tumor makes it challenging to conduct prospective clinical trials with sufficient power.¹⁰ As a result, most management recommendations are based on case series, retrospective reviews, and expert opinion.The pathogenesis, diagnosis, and staging of MCC are discussed in Part I of this review. Part II focuses on current management guidelines and promising new therapies in development. Due to the complexity, aggressive nature, and individuality of each case, MCC is best treated by a multidisciplinary team.

Teaser

Capsule Summary: word count not required for CME articles as these appear before each section and are included in the text word count

Dermoscopy as an evolving tool to assess vitiligo activity

Publication date: Available online 9 December 2017
Source:Journal of the American Academy of Dermatology
Author(s): Abhijeet Kumar Jha, Sidhath Sonthalia, Aimilios Lallas

Denitrifying haloarchaea: sources and sinks of nitrogenous gases

Abstract

Haloarchaea thrive under saline and hypersaline conditions and often dominate microbial communities in saltmarshes, salted lakes/soils, and some oceanic areas. Some of the predominant species show denitrifying capabilities, although it remains unclear whether they are complete or partial denitrifiers. As complete denitrifiers, they could play important roles buffering ecosystems in which nitrate and nitrite appear as contaminants. However, partial denitrifying haloarchaea could contribute to the emission of nitrogenous gasses, thus acting as drivers of climate change and ozone depletion. In this review, we summarise some recent results on denitrification in haloarchaea, discuss the environmental implications and outline possible applications in mitigation. Finally, we list questions to be addressed in the near future, facilitating increased understanding of the role of these organisms in N turnover in arid and hypersaline environments.

Belief dependence: How do the numbers count?

Abstract

This paper is about how to aggregate outside opinion. If two experts are on one side of an issue, while three experts are on the other side, what should a non-expert believe? Certainly, the non-expert should take into account more than just the numbers. But which other factors are relevant, and why? According to the view developed here, one important factor is whether the experts should have been expected, in advance, to reach the same conclusion. When the agreement of two (or of twenty) thinkers can be predicted with certainty in advance, their shared belief is worth only as much as one of their beliefs would be worth alone. This expectational model of belief dependence can be applied whether we think in terms of credences or in terms of all-or-nothing beliefs.

La « rupia syphilitique » existe toujours

Publication date: Available online 8 December 2017
Source:Annales de Dermatologie et de Vénéréologie
Author(s): J. Revuz

Quel avenir pour la dermatologie universitaire ?

Publication date: Available online 8 December 2017
Source:Annales de Dermatologie et de Vénéréologie
Author(s): B. Dréno, N. Dupin

Sclérodermie systémique de révélation tardive : étude rétrospective de 27 patients diagnostiqués après l’âge de 70 ans

Publication date: Available online 8 December 2017
Source:Annales de Dermatologie et de Vénéréologie
Author(s): A. Achille, L. Journeau, O. Espitia, J. Connault, A. Espitia-Thibault, C. Durant, F. Perrin, M.-A. Pistorius, A. Néel, M. Hamidou, C. Agard
IntroductionLe but de ce travail était de décrire les particularités de la sclérodermie systémique (ScS) quand le diagnostic est fait après l'âge de 70 ans.Patients et méthodesIl s'agit d'une étude rétrospective des patients âgés de plus de 70 ans au moment du diagnostic de ScS, suivis dans un service de médecine interne entre 2000 et 2015. Les co-morbidités et les caractéristiques démographiques et cliniques ont été analysées, ainsi que la survie à 1, 2 et 3 ans.RésultatsSur les 246 cas de ScS de notre cohorte, 27 (11 %) ont été inclus (89 % de femmes, âgés de 78,3±4,5 ans). Une néoplasie synchrone était notée chez 3 patients. La ScS était majoritairement cutanée limitée (24/27), avec des télangiectasies (63 %), un reflux gastro-oesophagien (59 %), des ulcères digitaux (22 %), et associée à un anticorps anti-centromères (69 % des cas). L'atteinte interstitielle pulmonaire était peu fréquente (29 %). Une hypertension artérielle pulmonaire (HTAP) était suspectée dès le diagnostic de ScS dans 14 cas (52 %), mais seuls 5 patients ont eu un cathétérisme cardiaque, montrant une HTAP sévère dans 3 cas. La survie était de 85,2 % à 1 an et 66,7 % à 3 ans ; elle était moins bonne en cas de suspicion d'HTAP (respectivement 78,6 % et 57,1 %).ConclusionLes cas de ScS diagnostiquée après 70 ans sont majoritairement des formes cutanées limitées. Une suspicion d'HTAP est fréquente, représentant plus d'un cas sur 2, et l'HTAP est un mode d'entrée dans la maladie à cet âge. L'association à un cancer synchrone est possible. La survie est médiocre.BackgroundThe aim of this study was to describe special features of patients with systemic sclerosis (SSc) diagnosed after the age of 70.Patients and methodsThis is a retrospective study of patients aged above 70 years at the time of diagnosis of SSc and followed at an internal medicine unit between 2000 and 2015. Co-morbidities and clinical characteristics were analyzed, as well as survival at 1, 2 and 3 years.ResultsOf 246 patients, 27 (11%) were included (89% women, 96% Caucasians, age 78.3±4.5 years). Synchronous cancer was noted in 3 patients. SSc was mostly limited cutaneous only (24/27), with telangiectasia (63%), gastroesophageal reflux (59%) and digital ulcers (22%), and was associated with anti-centromere antibody (69%). Interstitial lung disease was not frequent (29%). Pulmonary arterial hypertension (PAH) was suspected at diagnosis of SSc in 14 cases (52%), but only 5 patients had undergone heart catheterization, with severe PAH in 3 cases. Survival at 1 and 3 years was 85.2% and 66.7%, and was worse in the case of suspected PAH, at 78.6% and 57.1% respectively.ConclusionCases of SSc diagnosed after 70 years are mostly limited cutaneous forms. Suspicion of PAH is frequent, and PAH may be the main initial sign of the disease for patients at this age. There may be association with synchronous cancer. Survival is poor.