What causes hidradenitis suppurativa? - 15 years after

The 14 authors of the first review article on hidradenitis suppurativa (HS) pathogenesis published 2008 in EXPERIMENTAL DERMATOLOGY cumulating from the 1st International Hidradenitis Suppurativa Research Symposium held March 30?April 2, 2006 in Dessau, Germany with 33 participants were prophetic when they wrote "Hopefully, this heralds a welcome new tradition: to get to the molecular heart of HS pathogenesis, which can only be achieved by a renaissance of solid basic HS research, as the key to developing more effective HS therapy." (Kurzen et al. What causes hidradenitis suppurativa? Exp Dermatol 2008;17:455). Fifteen years later, there is no doubt that the desired renaissance of solid basic HS research is progressing with rapid steps and that HS has developed deep roots among inflammatory diseases in Dermatology and beyond, recognized as ?the only inflammatory skin disease than can be healed?. This anniversary article of 43 research-performing authors from all around the globe in the official journal of the European Hidradenitis Suppurativa Foundation e.V. (EHSF e.V.) and the Hidradenitis Suppurativa Foundation, Inc (HSF USA) summarizes the evidence of the intense HS clinical and experimental research during the last 15 years in all aspects of the disease and provides information of the developments to come in the near future

Human OTULIN haploinsufficiency impairs cell-intrinsic immunity to staphylococcal alpha-toxin

The molecular basis of interindividual clinical variability upon infection with Staphylococcus aureus is unclear. We describe patients with haploinsufficiency for the linear deubiquitinase OTULIN, encoded by a gene on chromosome 5p. Patients suffer from episodes of life-threatening necrosis, typically triggered by S. aureus infection. The disorder is phenocopied in patients with the 5p- (Cri-du-Chat) chromosomal deletion syndrome. OTULIN haploinsufficiency causes an accumulation of linear ubiquitin in dermal fibroblasts, but tumor necrosis factor receptor-mediated nuclear factor kappa B signaling remains intact. Blood leukocyte subsets are unaffected. The OTULIN-dependent accumulation of caveolin-1 in dermal fibroblasts, but not leukocytes, facilitates the cytotoxic damage inflicted by the staphylococcal virulence factor alpha-toxin. Naturally elicited antibodies against alpha-toxin contribute to incomplete clinical penetrance. Human OTULIN haploinsufficiency underlies life-threatening staphylococcal disease by disrupting cell-intrinsic immunity to alpha-toxin in nonleukocytic cells.Peer reviewe

What causes hidradenitis suppurativa ?—15 years after

The 14 authors of the first review article on hidradenitis suppurativa (HS) pathogenesis published 2008 in EXPERIMENTAL DERMATOLOGY cumulating from the 1st International Hidradenitis Suppurativa Research Symposium held March 30–April 2, 2006 in Dessau, Germany with 33 participants were prophetic when they wrote “Hopefully, this heralds a welcome new tradition: to get to the molecular heart of HS pathogenesis, which can only be achieved by a renaissance of solid basic HS research, as the key to developing more effective HS therapy.” (Kurzen et al. What causes hidradenitis suppurativa? Exp Dermatol 2008;17:455). Fifteen years later, th

Association of hidradenitis suppurativa and Down syndrome – a multicentre European study of 23 cases

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Superconductivity in the crystallogenide LaFeSiO1−δ with squeezed FeSi layers

International audiencePnictogens and chalcogens are both viable anions for promoting Fe-based superconductivity, and intense research activity in the related families has established a systematic correlation between the Fe-anion height and the superconducting critical temperature T c , with an optimum Fe-anion height of ~1.38 Å. Here, we report the discovery of superconductivity in the compound LaFeSiO 1− δ that incorporates a crystallogen element, Si, and challenges the above picture: considering the strongly squeezed Fe–Si height of 0.94 Å, the superconducting transition at T c = 10 K is unusually high. In the normal state, the resistivity displays non-Fermi-liquid behavior while NMR experiments evidence weak antiferromagnetic fluctuations. According to first-principles calculations, the Fermi surface of this material is dominated by hole pockets without nesting properties, which explains the strongly suppressed tendency toward magnetic order and suggests that the emergence of superconductivity materializes in a distinct set-up, as compared to the standard s ± - and d -wave electron-pocket-based situations. These properties and its simple-to-implement synthesis make LaFeSiO 1− δ a particularly promising platform to study the interplay between structure, electron correlations, and superconductivity

Association maladie de Verneuil et trisomie 21 – Étude internationale multicentrique de 56 cas

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Errance médicale des patients atteints d’hidradénite suppurée : un problème majeur et persistant. Étude « R-ENS Verneuil »

International audienceObjectiveTo provide physicians with an understanding of the factors behind significant delays in the diagnosis of hidradenitis suppurativa (HS) in France.Patients and methodsThis prospective multicentre national study conducted from October 2015 to March 2016 included all patients consulting for HS. Patient data were collected by means of a standardized questionnaire. Univariate and multivariate analyses were conducted to collect factors associated with a significant time to diagnosis of at least 5.5 years, defined as the period between the onset of initial clinical signs and the time of formal diagnosis.ResultsThe 16 participating centres enrolled 312 patients (62% women), of average age 35 years. The average age at onset of HS was 22 years. Before formal diagnosis by a dermatologist (64% of cases), 170 (54%), 114 (37%) and 45 (15%) patients had previously consulted at least 3, 5 and 10 general physicians, respectively. The average time between the initial clinical signs of HS, the first dermatology visit and the definitive diagnosis was 6.2 and 8.4 years, respectively. Active smoking (OR adjusted 1.85; P = 0.027) and disease onset at a younger age (adjusted OR 0.92; P < 0.001) were both associated with significant delays in diagnosis.ConclusionThese results emphasized misdiagnosis among HS patients but did not evidence any association between either sociodemographic or economic characteristics and the existence of significant times to diagnosis.ObjectifL’objectif principal de cette étude était d’analyser les facteurs associés à l’existence d’un délai diagnostique significatif chez les patients atteints d’hidradénite suppurée (HS) en France.Malades et méthodesCette étude nationale prospective multicentrique réalisée d’octobre 2015 à mars 2016 incluait tous les patients consultant pour une HS. Les données concernant les patients étaient recueillies à l’aide d’un questionnaire standardisé. Des analyses univariée et multivariée ont été effectuées afin de recueillir les facteurs associés à un délai diagnostique significatif, défini comme un délai d’au moins 5,5 ans entre les premiers signes de la maladie et son diagnostic formel.RésultatsLes 16 centres ont inclus 312 patients (62 % de femmes) d’âge moyen 35 ans. L’âge moyen de début de l’HS était de 22 ans. Respectivement 170 (54 %), 114 (37 %) et 45 (15 %) patients avaient consulté au moins 3, 5 et 10 médecins avant le diagnostic formel, qui était posé par un dermatologue dans 64 % des cas. Les premiers signes d’HS étaient apparus en moyenne 6,2 ans avant la première consultation de dermatologie et 8,4 ans avant le diagnostic. Un tabagisme actif (Odd Ratio (OR) ajusté 1,85; p = 0,027) et un début des signes à un âge plus précoce (OR ajusté 0,92; p < 0,001) étaient associés à un délai diagnostique significatif.ConclusionCes résultats soulignent l’errance médicale des patients atteints d’HS, mais ne montrent pas d’association entre les caractéristiques sociodémographiques ou économiques des patients et l’existence d’un délai diagnostique significatif

Proceeding report of the Second Symposium on Hidradenitis Suppurativa Advances (SHSA) 2017

The 2nd Annual Symposium on Hidradenitis Suppurativa Advances (SHSA) took place on 03-05 November 2017 in Detroit, Michigan, USA. This symposium was a joint meeting of the Hidradenitis Suppurativa Foundation (HSF Inc.) founded in the USA, and the Canadian Hidradenitis Suppurativa Foundation (CHSF). This was the second annual meeting of the SHSA with experts from different disciplines arriving from North America, Europe and Australia, in a joint aim to discuss most recent innovations, practical challenges and potential solutions to issues related in the management and care of Hidradenitis Suppurativa patients. The last session involved clinicians, patients and their families in an effort to educate them more about the disease

Evaluating patients' unmet needs in hidradenitis suppurativa: Results from the Global Survey Of Impact and Healthcare Needs (VOICE) Project

Background: A needs assessment for patients with hidradenitis suppurativa (HS) will support advancements in multidisciplinary care, treatment, research, advocacy, and philanthropy. Objective: To evaluate unmet needs from the perspective of HS patients. Methods: Prospective multinational survey of patients between October 2017 and July 2018. Results: Before receiving a formal HS diagnosis, 63.7% (n = 827) of patients visited a physician ≥5 times. Mean delay in diagnosis was 10.2 ± 8.9 years. Patients experienced flare daily, weekly, or monthly in 23.0%, 29.8%, and 31.1%, respectively. Most (61.4% [n = 798]) rated recent HS-related pain as moderate or higher, and 4.5% described recent pain to be the worst possible. Access to dermatology was rated as difficult by 37.0% (n = 481). Patients reported visiting the emergency department and hospital ≥5 times for symptoms in 18.3% and 12.5%, respectively. An extreme impact on life was reported by 43.3% (n = 563), and 14.5% were disabled due to disease. Patients reported a high frequency of comorbidities, most commonly mood disorders. Patients were dissatisfied with medical or procedural treatments in 45.9% and 34.6%, respectively. Limitations: Data were self-reported. Patients with more severe disease may have been selected. Conclusion: HS patients have identified several critical unmet needs that will require stakeholder collaboration to meaningfully address