L’efficacité clinique de la ventilation non invasive de longue durée (VNILD) est à ce jour démontrée. Or la VNILD s’applique à des pathologies différentes et hétérogènes en termes de physiopathologie, de mécanique ventilatoire et d’évolution. La cohorte ANTADIR-GAVO2 a comme objectif d’analyser prospectivement les données des patients relevant d’une VNILD en France. L’objectif de cette étude est de recenser les spécificités des patients selon les différentes étiologies d’insuffisance respiratoire chronique (IRC).

Bien que l’efficacité clinique de la ventilation non invasive (VNI) soit clairement démontrée, il n’existe aucune étude récente à propos des conditions de prescription et d’utilisation de celle-ci selon les différentes pathologies respiratoires. La cohorte ANTADIR–GAV a pour objectif d’analyser prospectivement les données en France des patients relevant d’une VNI à domicile. Étude multicentrique prospective et observationnelle. L’étiologie de l’insuffisance respiratoire chronique (IRC), les données démographiques, les comorbidités, les gaz du sang (GDS), les données fonctionnelles respiratoires, le type de ventilateur, le mode, les paramètres ventilatoires et les interfaces ont été collectées. De mai 2015 à décembre 2016, 964 patients (54 % hommes, âge 64 ± 14 ans, IMC 28 ± 10 kg/m2) ont été inclus dans 19 centres. L’IRC était d’origine neuromusculaire chez 51 % des patients, parenchymateuse chez 25 % ou due à une pathologie de la cage thoracique chez 24 %. Les étiologies les plus fréquentes étaient : sclérose latérale amyotrophique (36 %), BPCO (22 %) et SOH (19 %). Les principales comorbidités étaient : hypertension (40 %), diabète (18 %), arythmie (13 %) et maladie coronaire (8 %). Les GDS avant VNI étaient : PaO2 73 ± 14 et PaCO2 49 ± 8 mmHg. La majorité des patients a été ventilée par un ventilateur monobranche avec fuite (99 %). Au total, 83 % des patients étaient ventilés en mode ST, 10 % en mode S et 4 % avec un mode hybride. Chez 892 patients (89,4 %) un ventilateur à double niveau de pression a été utilisé, sans batterie chez 47 % des patients ou avec batterie chez 45 %. Un ventilateur support de vie a été nécessaire chez 8 % des patients. L’IPAP, l’EPAP et la fréquence de sécurité ont été respectivement 16 ± 4 cmH2O, 7 ± 3 cmH2O et 15 ± 3 c/min. L’interface la plus utilisée a été un masque oronasal (80 % des patients). L’adjonction d’O2 a été nécessaire chez 32 % des patients. La VNI a été mise en route après un épisode aigu chez 43 % des patients (26 % en réanimation, 23 % en SI de pneumologie et 50 % en salle de pneumologie) et à l’état stable chez 57 % (58 % en hospitalisation conventionnelle, 36 % en hôpital de jour, 6 % à domicile), Les critères principaux pour l’initiation de la VNI ont été les GDS (51,8 % des patients), les symptômes (33,3 %) et les explorations nocturnes (8,9 %). Cette cohorte prospective offre une analyse fiable et représentative de la réalité de la VNI à domicile en France et ouvre de nouvelles perspectives pour l’organisation de sa prise en charge et la recherche clinique.

Introduction. Noninvasive ventilation (NIV) is considered as the first choice treatment for selected patients with acute respiratory failure (ARE), but many hospitals are forced to start NIV on medical wards.Methods. The aim of this retrospective study was to assess the outcomes of NIV initiated for ARE on a respiratory ward and to find the criteria predictive of failure. All patients were treated in a four-bed ward specifically dedicated to NIV. Failure of NIV was defined as the need for intubation and transfer to ICU, or death.Results. Among 105 admissions with ARF, 49 episodes needed NIV. These episodes were divided into 2 groups: PaCO2 < 45 mmHg (10) and PaCO2 > 45 mmHg (39). The overall failure rate of NIV and overall in-hospital mortality rate were 26.5% and 17% respectively. On multivariate analysis, SAPS II and respiratory acidosis with a pH less than 7.30 were significantly associated with failure of NIV.Conclusions. NIV is practicable and is effective in the management of mild to moderate ARE on a respiratory ward. However, patients with respiratory acidosis and a pH less than 7.30 are at risk of NIV failure. (C) 2015 SPLF. Published by Elsevier Masson SAS. All rights reserved.

La sclérose latérale amyotrophique est une affection dégénérative caractérisée par une dégénérescence du neurone moteur central et périphérique dans le territoire bulbaire et spinal. Le diagnostic en est souvent difficile en raison de l’hétérogénéité phénotypique et surtout de l’absence de marqueur paraclinique qui signerait l’affection. Dans la mesure où il est apparu indispensable d’établir des critères diagnostiques, trois conférences de consensus se sont tenues au cours des 20 dernières années. La première classification reposait sur l’existence de signes cliniques d’atteinte du NMc et du NMp au sein du système nerveux décomposé en quatre régions anatomiques. Compte tenu de la faible sensibilité de ces critères initiaux deux autres conférences ont eu lieu ensuite. La dernière qui s’est déroulée à Awaji en 2006 a insisté sur l’importance des données myographiques qui apportaient des informations semblables à la clinique pour dépister une atteinte du NMp ainsi que sur l’importance des fasciculations dans le diagnostic de SLA. Nous discuterons de l’intérêt et des limites de ces nouveaux critères dans le diagnostic de SLA.Amyotrophic lateral sclerosis is the most common motor neuron disorder in adults. Although the diagnosis appears obvious in theory, clinical practice shows the contrary as diagnosis is delayed in many patients; the average time between symptom onset and diagnosis can reach 12 months. The delay can be explained by the variability of the clinical presentation and by the absence of diagnostic markers. In order to standardize diagnosis for enrolment in clinical research, diagnostic criteria for ALS were created and revisited during the last 20 years. In 2006, the Awaji criteria for the diagnosis of ALS were proposed, adding two major points to the diagnostic criteria: electromyography is considered equivalent to clinical examination for the identification of LMN signs and fasciculation potentials resume their prominent place in the diagnosis. Comparisons of the accuracy of the revisited El Escorial and Awaji criteria support improved diagnostic sensitivity without any effect on specificity with the new classification. The only weakness of the new classification involves patients with UMN signs in one region and LMN in two regions; these patients were previously classified as laboratory-supported probable ALS and currently as possible ALS, a lower level of diagnostic certainty. In all other instances the accuracy appears to be improved by the Awaji criteria. Nevertheless, there is a body of evidence suggesting the need for a revision of these new criteria, giving more weight to clinical and complementary findings of UMN involvement. The need to diagnose and treat ALS quickly could be facilitated by the inclusion of complementary investigations that detect UMN signs.

BACKGROUND:Blastic plasmacytoid dendritic cell neoplasm (BPDCN), formerly known as agranular CD4(+) /CD56(+) haematodermic neoplasm (CD4/CD56 HN), is a rare distinct form of lymphoma-like entity known of dermatologists because of its marked predilection for cutaneous involvement, and its aggressive behaviour. Moreover, the association or the evolution to an acute leukaemia entity that still expresses CD4 and CD56 markers is almost systematic. This new described entity of 'CD4(+) /CD56(+) leukaemia' or 'leukaemia of plasmacytoid dendritic cell lineage' has a poor prognostic and may lead to include haematopoietic stem cell transplantation in the treatment strategy as early as possible.REPORT OF CASES:We report here four cases presenting with skin lesions and haematological signs. One of the patients underwent allogeneic stem cell transplantation, with a relapse-free survival of 40 months. We discuss the diagnosis features as well as the treatment options.CONCLUSION:A collaborative work between dermatologists and onco-haematologists is essential to give patients the best chance of complete and long-term response.

La peau est soumise continuellement au rayonnement solaire qui induit des dommages directs et indirects, via la production d’espèces réactives d’oxygène, sur les constituants majeurs des cellules, en particulier l’acide désoxyribonucléique, à l’origine des effets biologiques et cliniques du soleil sur la peau. Il est classique de les classer en fonction de leur apparition chronologique. L’action calorique est le fait des infrarouges. La synthèse de vitamine D débute dans la peau en nécessitant des doses faibles d’ultraviolets B (UVB). La pigmentation immédiate, liée à l’oxydation des prémélanines n’a pas de rôle biologique reconnu. L’érythème dépend de l’intensité de l’exposition, mais aussi du phototype de l’individu; son marqueur histologique est la « sunburn cell », kératinocyte en apoptose. Les UVB sont les longueurs d’onde les plus efficaces, mais les UVA y participent aussi. Ses mécanismes relèvent principalement d’une libération de nombreux médiateurs de l’inflammation par les kératinocytes (principalement des prostaglandines) et par les mastocytes (histamine). Le bronzage est déclenché par les UV et la lumière bleue. Son mécanisme est différent selon la longueur d’onde déclenchante; dans les phototypes clairs, il n’apparaît pas comme un effet positif et adaptatif. La photo-immunosuppression relève d’une orientation de la réponse à l’antigène vers une tolérance spécifique. La cellule de Langerhans a un rôle central, ainsi que le kératinocyte qui libère des cytokines immunosuppressives, principalement l’interleukine 10. L’héliodermie, conséquence des expositions chroniques, a pour expression fondamentale l’élastose solaire. Les espèces réactives d’oxygène ont un rôle déterminant dans sa genèse. Concernant la cancérogenèse cutanée, le rôle des UV est clairement établi pour les carcinomes, moins directement évident pour les mélanomes. Tous les UV participent à la photocarcinogenèse et interviennent à tous ses stades. Les dommages directs à l’ADN ont un rôle majeur dans les carcinomes et portent sur les gênes suppresseurs de tumeurs; les dommages oxydatifs pourraient avoir un rôle-clé dans les mélanomes. La photo-immunosuppression a aussi un rôle important dans la promotion tumorale des cancers cutanés.The skin is continuously subjected to solar radiation which induces direct and indirect damage, via the production of reactive oxygen species, on the major constituents of the cells, in particular deoxyribonucleic acid, at the origin of the biological and clinical effects of the sun on the skin. It is traditional to classify them according to their chronological appearance. The caloric action is due to infrared radiation. Vitamin D synthesis begins in the skin by requiring low doses of ultraviolet B (UVB). Immediate pigmentation, related to the oxidation of premelanins, has no recognised biological role. The erythema depends on the intensity of exposure, but also on the phototype of the individual; its histological marker is the “sunburn cell”, a keratinocyte in apoptosis. UVB is the most effective wavelength, but UVA is also involved. Its mechanisms are mainly related to the release of numerous inflammatory mediators by keratinocytes (mainly prostaglandins) and by mast cells (histamine). Tanning is triggered by UV and blue light. Its mechanism is different depending on the triggering wavelength; in light phototypes it does not appear as a positive, adaptive effect. Photo-immunosuppression is a matter of orienting the antigen response towards specific tolerance. The Langerhans cell has a central role, as does the keratinocyte which releases immunosuppressive cytokines, mainly interleukin 10. Helioderma, a consequence of chronic exposure, has as its fundamental expression solar elastosis. Reactive oxygen species play a determining role in its genesis. Concerning cutaneous carcinogenesis, the role of UV is clearly established for carcinomas, less directly evident for melanomas. All UV rays participate in photocarcinogenesis and are involved at all stages. Direct DNA damage has a major role in carcinomas and affects tumour suppressor genes; oxidative damage may have a key role in melanomas. Photo-immunosuppression also has an important role in the tumour promotion of skin cancers.

BACKGROUND:Scleromyxoedema is a rare disorder of unknown pathogenesis that is very difficult to treat. We report a case resistant to corticosteroid treatment but controlled by intravenous gammaglobulin (IVIG). CASE REPORT:A 60-year-old woman presented progressive generalized papular eruption with infiltrated and itchy lesions of between 2 and 5mm in diameter. Otherwise, the clinical examination was normal. Monoclonal gammopathy of the IgG lambda type was found. Histology confirmed the diagnosis of scleromyxoedema. The disease continued to progress despite oral corticosteroids (0.5mg/kg per day). Thalidomide was introduced but was discontinued after 2 months due to side effects. Treatment comprising six monthly infusions of IVIG (2g/kg on 5 days) resulted in a marked reduction (>50%) in lesions. Two months after discontinuation of IVIG, recurrence was observed and maintenance infusions of IVIG every 6 weeks were needed to control the disease. DISCUSSION:The course of scleromyxoedema is unpredictable and treatment is extremely difficult. Successful therapy with IGIV has been reported but this approach seems to afford only temporary relief and maintenance infusions are required, as confirmed by the initial efficacy of treatment in our patient and the rapid recurrence of lesions following withdrawal.

Les tumeurs à cellules géantes (TCG) synoviales sont des tumeurs bénignes d’origine synoviale qui peuvent intéresser les synoviales articulaires, les bourses séreuses et les gaines tendineuses. Elles s’observent le plus souvent au niveau des mains où elles représentent la seconde tumeur des parties molles après le kyste synovial. La forme localisée est la plus fréquente (ténosynovite nodulaire). Dans la forme diffuse, la TCG représente l’équivalent dans la gaine tendineuse ou la bourse séreuse de la synovite villonodulaire pigmentée articulaire. Les auteurs rapportent un cas d’une tumeur ténosynoviale à cellules géantes diffuse qui s’est développée aux dépens du premier compartiment du poignet et qui a envahi le canal carpien en refoulant les tendons fléchisseurs et comprimant le nerf médian. La patiente a consulté initialement pour un syndrome du canal carpien associé à une masse au niveau du poignet qui augmentait progressivement de volume. Nous discutons le caractère inhabituel de la localisation, l’intérêt et la difficulté d’une exérèse large de ce type de lésion redoutée par les risques élevés de récidives.Giant cell tumour of tendon sheath is a benign proliferative lesion of synovial origin that may affect the joints, bursae and tendon sheaths. It is the second most common soft tissue tumor of the hand after ganglion cyst. The localised (nodular) form is the most common. However, the less-common diffuse-type giant cell tumour is usually located in the peri-articular soft tissue. The authors report the case of a giant cell tumor of the tendon sheath arising from the carpal tunnel of the wrist in a 42-year-old woman. The patient presented a mild carpal tunnel syndrome and a mid-palmar swelling. We present an unusual localization of giant cell tumor of the tendon sheath, causing carpal tunnel syndrome.

HistopathologyVolume 53, Issue 3 p. 361-363 Cutaneous myxolipoma with apocrine glandular differentiation: description of a new clinicopathological variant with chromosome 6p21 rearrangement N Cardot-Leccia, N Cardot-Leccia Department of PathologySearch for more papers by this authorA Italiano, A Italiano Laboratory of Solid Tumour Genetics, Nice University Hospital CNRS UMR 6543, Faculty of MedicineSearch for more papers by this authorJ Haudebourg, J Haudebourg Department of PathologySearch for more papers by this authorR Attias, R Attias Laboratory of Solid Tumour Genetics, Nice University Hospital CNRS UMR 6543, Faculty of MedicineSearch for more papers by this authorD Amato, D Amato Department of Digestive Surgery, Nice University Hospital, Nice, FranceSearch for more papers by this authorF Pedeutour, F Pedeutour Laboratory of Solid Tumour Genetics, Nice University Hospital CNRS UMR 6543, Faculty of MedicineSearch for more papers by this authorC Perrin, C Perrin Department of PathologySearch for more papers by this author N Cardot-Leccia, N Cardot-Leccia Department of PathologySearch for more papers by this authorA Italiano, A Italiano Laboratory of Solid Tumour Genetics, Nice University Hospital CNRS UMR 6543, Faculty of MedicineSearch for more papers by this authorJ Haudebourg, J Haudebourg Department of PathologySearch for more papers by this authorR Attias, R Attias Laboratory of Solid Tumour Genetics, Nice University Hospital CNRS UMR 6543, Faculty of MedicineSearch for more papers by this authorD Amato, D Amato Department of Digestive Surgery, Nice University Hospital, Nice, FranceSearch for more papers by this authorF Pedeutour, F Pedeutour Laboratory of Solid Tumour Genetics, Nice University Hospital CNRS UMR 6543, Faculty of MedicineSearch for more papers by this authorC Perrin, C Perrin Department of PathologySearch for more papers by this author First published: 20 August 2008 https://doi.org/10.1111/j.1365-2559.2008.03085.xCitations: 3Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat Citing Literature Volume53, Issue3September 2008Pages 361-363 RelatedInformation

Background. Mycosis fungoides is a cutaneous T-cell lymphoma. The early stages have a good prognosis but pose therapeutic problems due to chronic disease status and the frequent recurrence of lesions. We performed a retrospective clinical and histological study in 10 patients presenting mycosis fungoides in order to evaluate the efficacy of 308 nm excimer laser in this indication. Methods. Ten patients with mycosis fungoides confirmed by histological examination were included. Treatment by 308 nnn excimer laser was performed on a total of 29 lesions (25 patch-stage, 3 plaques and 1 nodule). After determination of the minimal erythernal dose, sessions were started twice weekly gradually increasing in accordance with tolerability. Clinical follow-up was performed and histological and immunohistological analysis was done, if possible, before and after treatment and at the end of the follow-up. Results. Eighty-six percent of patch-stage or plaque lesions had completely cleared up by the end of treatment and 14% had cleared up partially, with a global response rate of 100%. The mean number of sessions was 15 (6 to 46 sessions) corresponding to a mean treatment duration of 2 months. The mean cumulative dose was 5 J/cm(2) (1.3 to 16.1 cm(2)). Follow-up was performed in 19 lesions for a median period of 15 months (8 to 26 months). Persistent complete clearance was observed in 13 of 19 (68%) lesions (12 patch-stage and 1 plaque). Continued partial clearance was noted in 3 lesions (1 patch-stage and 2 plaques) (16%). Two lesions (both patch-stage) (11%) showed relapse 7 months after the end of treatment. The sole nodule in the study showed no response to treatment. Histological and immunohistological were consistent with clinical results and showed histological healing where lesions had clinically disappeared, except for one case with persistence of a few mycosis cells in the epidermis despite a clinical appearance of healing. Discussion. This study demonstrates the efficacy of 308 nm excinner laser in stage la of mycosis fungoides. Given its limited availability and high cost, 308 nm excimer laser may be used as second-line therapy after failure of treatment with topical steroids.

BACKGROUND:Mycosis fungoides is a cutaneous T-cell lymphoma. The early stages have a good prognosis but pose therapeutic problems due to chronic disease status and the frequent recurrence of lesions. We performed a retrospective clinical and histological study in 10 patients presenting mycosis fungoides in order to evaluate the efficacy of 308 nm excimer laser in this indication.METHODS:Ten patients with mycosis fungoides confirmed by histological examination were included. Treatment by 308 nm excimer laser was performed on a total of 29 lesions (25 patch-stage, 3 plaques and 1 nodule). After determination of the minimal erythemal dose, sessions were started twice weekly gradually increasing in accordance with tolerability. Clinical follow-up was performed and histological and immunohistological analysis was done, if possible, before and after treatment and at the end of the follow-up.RESULTS:Eighty-six percent of patch-stage or plaque lesions had completely cleared up by the end of treatment and 14% had cleared up partially, with a global response rate of 100%. The mean number of sessions was 15 (6 to 46 sessions) corresponding to a mean treatment duration of 2 months. The mean cumulative dose was 5 J/cm2 (1.3 to 16.1 cm2). Follow-up was performed in 19 lesions for a median period of 15 months (8 to 26 months). Persistent complete clearance was observed in 13 of 19 (68%) lesions (12 patch-stage and 1 plaque). Continued partial clearance was noted in 3 lesions (1 patch-stage and 2 plaques) (16%). Two lesions (both patch-stage) (11%) showed relapse 7 months after the end of treatment. The sole nodule in the study showed no response to treatment. Histological and immunohistological were consistent with clinical results and showed histological healing where lesions had clinically disappeared, except for one case with persistence of a few mycosis cells in the epidermis despite a clinical appearance of healing.DISCUSSION:This study demonstrates the efficacy of 308 nm excimer laser in stage Ia of mycosis fungoides. Given its limited availability and high cost, 308 nm excimer laser may be used as second-line therapy after failure of treatment with topical steroids.

Journal Article Naevus lipomatosus superficialis: a case report with a 2p24 deletion Get access N. Cardot‐Leccia, N. Cardot‐Leccia Department of Pathology, Pasteur Hospital, 20 avenue de la Voie Romaine, 06002 Nice, France *Laboratory of Solid Tumour Genetics, University Hospital and CNRS UMR 6543, Faculty of Medicine, Nice, France †Department of Plastic Surgery, Saint‐Roch Hospital, Nice, France Search for other works by this author on: Oxford Academic Google Scholar A. Italiano, A. Italiano Department of Pathology, Pasteur Hospital, 20 avenue de la Voie Romaine, 06002 Nice, France *Laboratory of Solid Tumour Genetics, University Hospital and CNRS UMR 6543, Faculty of Medicine, Nice, France †Department of Plastic Surgery, Saint‐Roch Hospital, Nice, France Search for other works by this author on: Oxford Academic Google Scholar M.C. Monteil, M.C. Monteil Department of Pathology, Pasteur Hospital, 20 avenue de la Voie Romaine, 06002 Nice, France *Laboratory of Solid Tumour Genetics, University Hospital and CNRS UMR 6543, Faculty of Medicine, Nice, France †Department of Plastic Surgery, Saint‐Roch Hospital, Nice, France Search for other works by this author on: Oxford Academic Google Scholar E. Basc, E. Basc Department of Pathology, Pasteur Hospital, 20 avenue de la Voie Romaine, 06002 Nice, France *Laboratory of Solid Tumour Genetics, University Hospital and CNRS UMR 6543, Faculty of Medicine, Nice, France †Department of Plastic Surgery, Saint‐Roch Hospital, Nice, France Search for other works by this author on: Oxford Academic Google Scholar C. Perrin, C. Perrin Department of Pathology, Pasteur Hospital, 20 avenue de la Voie Romaine, 06002 Nice, France *Laboratory of Solid Tumour Genetics, University Hospital and CNRS UMR 6543, Faculty of Medicine, Nice, France †Department of Plastic Surgery, Saint‐Roch Hospital, Nice, France Correspondence: Christophe Perrin. E‐mail: xst.perrin@wanadoo.fr Search for other works by this author on: Oxford Academic Google Scholar F. Pedeutour F. Pedeutour Department of Pathology, Pasteur Hospital, 20 avenue de la Voie Romaine, 06002 Nice, France *Laboratory of Solid Tumour Genetics, University Hospital and CNRS UMR 6543, Faculty of Medicine, Nice, France †Department of Plastic Surgery, Saint‐Roch Hospital, Nice, France Search for other works by this author on: Oxford Academic Google Scholar British Journal of Dermatology, Volume 156, Issue 2, 1 February 2007, Pages 380–381, https://doi.org/10.1111/j.1365-2133.2006.07622.x Published: 01 February 2007

Respiratory muscle weakness represents the major cause of mortality in patients with amyotrophic lateral sclerosis (ALS). As a result, ventilatory assistance is an important part of disease management. Nowadays, noninvasive ventilation (NIV) has become the first choice modality for most patients and represents an alternative to tracheostomy intermittent positive-pressure ventilation. Although, some consensus guidelines have been proposed to initiate NIV in patients with restrictive chronic respiratory failure, these criteria are discussed regarding ALS. While the current consensus recommends that NIV may be used in symptomatic patients with hypercapnia or forced vital capacity < 50p.cent of predicted value, early use of NIV is proposed in the literature and reported in this paper.

Although noninvasive ventilation may improve survival in patients with amyotrophic lateral sclerosis (ALS), ineffective airway clearance is an important cause of therapeutic failure. We report in this paper the main studies which have assessed assisted cough techniques in patients with ALS. Manually assisted cough (in particular with previous air stacking) and mechanical insufflation/exsufflation may significantly increase cough peak flow. Characteristics, limitations and long-term benefits of these techniques are also discussed.