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Subcorneal pustular dermatosis (SPD), also known as Sneddon-Wilkinson disease, is a skin condition for which treatments are poorly codified. Anti-tumor necrosis factor alpha (TNFα) efficacy has been reported in multidrug-resistant SPD, as in our two cases. In the first case, an 83-year-old woman was monitored for SPD, associated with monoclonal IgA gammopathy. After multiple-line treatment failure, infliximab (5mg/kg) led to clinical improvement, noted few days following the first injection, and with complete remission at one month. At 12 months, the patient relapsed and concomitant serum anti-TNFα antibodies were found. A switch to adalimumab led to complete remission in three months with a follow-up of six months. In the second case, a 62-year-old woman was monitored for SPD associated with monoclonal IgA gammopathy recalcitrant to different lines of treatment. Treatment with adalimumab (40mg every two weeks) in combination with dapsone led to significant improvement after two injections. Five months later, she relapsed. It was then decided to reduce the interval between injections to once a week. Rapid improvement was achieved in one month allowing resumption of the original frequency of the injection without relapse after 20 months of follow-up. In conclusion, our cases confirm the previously reported efficacy of anti-TNFα in resistant SPD. They also highlight a risk of secondary loss of efficacy, reinforced by the literature data. Substitution of another TNFα blocker or shortening of interval between injections provided a renewal in response to treatment.

International audience; This retrospective observational study aimed to determine the effectiveness, safety and patterns of the use of nivolumab in patients with advanced melanoma in real-world clinical practice in France using data from a Temporary Authorization for Use Program (ATU). Data were collected from patients with unresectable or metastatic melanoma enrolled in a French national database (Réseau pour la Recherche et l'Investigation Clinique sur le Mélanome: Ric-Mel) and treated with nivolumab during the ATU program (12 September 2014 to 31 August 2015). The primary objectives of the study were to evaluate the effect of patient characteristics on clinical response and overall survival (OS). Among 400 included patients (median age 66 years), the majority (83%) received nivolumab as second- or subsequent-line therapy. The median durations of progression-free survival and OS were 3.3 and 14.1 months, respectively, and 31.6% of patients achieved an objective response with a median duration of 20.1 months (range: 0-34.7). The safety profile of nivolumab was manageable and consistent with those of previous clinical trials, with an incidence of grade 3-5 adverse events of 13.8%. The safety and effectiveness of nivolumab in patients with advanced melanoma in real-world clinical practice in France were in line with the data reported in the Phase 3 trials CheckMate 066 and 037 of nivolumab in this patient population.

International audience; Since the coronavirus disease 2019 (COVID 19) outbreak was first reported in the Chinese city of Wuhan on December 31, 2019, it has stricken more than 1,000,000 persons worldwide, of whom over 50,000 have died (1). Having been infected by severe acute respiratory syndrome coronavirus 2 (SARS‐COV‐2), patients with COVID‐19 mainly present with fever and respiratory symptoms (2). Isolated sudden onset anosmia has also frequently been reported (3). Less frequently, rhinorrhea, diarrhoea and dysgeusia may be associated. While only a few reports have evoked cutaneous manifestations (4), we read with interest an initial study on the topic entitled “Cutaneous manifestations in COVID‐19: a first perspective ” by Recalcati S. (5).

International audience; BACKGROUND: Extra-mammary Paget's disease is a rare form of intraepithelial adenocarcinoma with a variable pattern of invasion, sometimes associated with distant malignancy. Vulvar Paget's disease (VPD) represents 1% of all vulvar cancers. Standard treatment is surgical excision, however, the recurrence rate is high and surgery leads to anatomical, functional, and sexual morbidity. Some case series suggest efficacy of photodynamic (PDT) as treatment for this indication.OBJECTIVES: Our aim was to assess the efficacy and tolerance of PDT in women suffering from VPD.MATERIALS & METHODS: A retrospective study was conducted on all patients suffering from VPD, treated with PDT at six hospitals in France. Clinical data, histopathological reports at diagnosis, therapeutic history, PDT schedule, tolerance, and clinical response were reported.RESULTS: Thirteen women were included, with a mean age of 70.1 years. Ten women had an in situ disease, two had CONCLUSION: PDT appears to be a therapeutic option for patients with VPD, with a response rate similar to that of other therapies usually recommended, such as surgery and imiquimod. There is a need to harmonise PDT procedures and pain assessment.

Introduction L’epidemie de COVID-19 a debute en decembre 2019 a Wuhan (Chine). Les patients infectes presentent principalement les symptomes suivants : symptomes respiratoires, fievre, rhinorrhee. Des episodes de diarrhees, d’anosmie et d’agueusie ont pu etre observes a plus faible frequence. Recemment, une etude italienne rapportait que 20 % des patients hospitalises pour COVID-19 presentaient une atteinte cutanee. L’objectif est de demontrer l’incidence des manifestations cutanees en France chez les patients atteints de COVID-19 et le type de reaction. Materiel et methodes Etude observationnelle prospective realisee entre le 15 mars et le 2 avril 2020 chez des patients dont le diagnostic de COVID-19 etait confirme par PCR nasopharyngee. Resultats Cent trois patients ont ete inclus dans cette etude : 71 femmes et 32 hommes avec une moyenne d’âge de 47 ans (20-88 ans). Parmi ces patients, 76 etaient traites en ambulatoire, 23 en hospitalisation conventionnelle et 4 etaient admis en reanimation. Aucun ne decedait au cours de l’etude. Seulement 5 patients (4,9 %) presentaient des manifestations cutanees. Deux eruptions erythemateuses maculopapuleuses et 2 urticaires, principalement localisees sur le visage et le haut du corps etaient observees, ainsi qu’une reactivation d’herpes oral chez un patient en reanimation. Discussion En France, au debut de la pandemie, seuls les patients ayant des symptomes respiratoires graves etaient depistes. Il est donc difficile de determiner une veritable incidence de l’infection et donc l’incidence des manifestations cutanees. C’est pourquoi l’incidence observee est minime par rapport a l’etude de Recalcati S en Italie. Depuis avril 2020, plusieurs etudes, dont une realisee par la SFD, ont pu montrer qu’il existe surtout des exanthemes maculopapuleux, des urticaires et des livedos chez des patients ayant une infection a SARS-Cov-2 confirmee ou suspectee. Finalement, l’incidence des eruptions cutanees semble faible, dans une revue de la litterature (Paulo Ricardo Criado et al.) realisee debut juin, on ne retrouve pas plus de 600 cas rapportes de manifestations cutanees sur plus 4 millions de patients atteints de la COVID-19. Ce faible nombre peut aussi etre explique par une sous notification des cas de manifestations cutanees devant la gravite moindre. De plus, plusieurs cas d’engelures, inhabituellement frequentes en cette saison, ont ete rapportes par des dermatologues ou generalistes. Cette forme survient plutot chez les jeunes et sans aucun signe infectieux associe dans la majorite des cas contrairement aux eruptions cutanees non specifiques. Cependant, plusieurs patients atteints d’engelures ont ete testes par PCR et le test etait majoritairement negatif. Le recours aux tests serologiques pourrait permettre d’etablir un lien ou non, surtout s’il s’agit d’une manifestation post infectieuse.

Introduction Le dermatoneuro syndrome est une complication neurologique particuliere du scleromyx�deme, rapportee une vingtaine de fois dans la litterature medicale. Il associe fievre, coma, convulsions et syndrome pseudo-grippal. Le devenir des patients atteints est mal connu. Nous decrivons l�observation d�une patiente ayant repondu favorablement a l�association de plasmaphereses et d�immunoglobulines intraveineuses (IgIV). Observation Une femme de 57 ans etait atteinte de scleromyx�deme evoluant depuis 14 ans, resistant a de multiples therapeutiques. En novembre 2011, elle presentait un premier episode de crises convulsives suivi d�un coma post-critique, puis d�un syndrome confusionnel associe a des hallucinations visuelles, spontanement resolutif en quelques jours. La recherche d�une infection etait negative et l�imagerie cerebrale (tomodensitometrie et IRM) ainsi que l�electroencephalogramme etaient parfaitement normaux, soulevant l�hypothese d�une atteinte neurologique de son scleromyx�deme. En decembre 2012 et aout 2013, elle presentait deux nouveaux episodes d�etat de mal epileptique, suivis a nouveau d�un etat confusionnel, pour lequel la recherche etiologique etait encore negative. Cette fois, l�etat confusionnel se perennisait pendant deux mois, jusqu�a la mise en place des echanges plasmatiques, suivie d�une cure d�IgIV. Cette association therapeutique permettait d�obtenir une regression rapide de l�ensemble des symptomes neurologiques et une amelioration de l�etat general. Discussion Le dermatoneuro syndrome est une complication neurologique centrale rare du scleromyx�deme. Sa physiopathologie est inconnue. La gammapathie monoclonale associee au scleromyx�deme pourrait expliquer un etat d�hypercoagulabilite ou la formation d�agregats neutrophiliques aboutissant a une alteration de la microcirculation. Le traitement est empirique et mal codifie. L�evolution est imprevisible et potentiellement letale. Summary Background Dermato-neuro syndrome is a specific neurological complication of scleromyxedema presenting with fever, coma, seizures and flu-like syndrome. To our knowledge, it has only been reported about twenty times in the literature. Its outcome is uncertain. We describe the case of a patient in whom a favorable outcome was achieved using a combination of plasmapheresis and intravenous immunoglobulin (IVIG). Patients and methods A 57-year-old woman was diagnosed 14 years ago with scleromyxedema resistant to multiple lines of treatment. In November 2011, she presented an initial episode of epileptic seizure followed by post-seizure coma, and later, confusional state with visual hallucinations. She recovered spontaneously within a few days. CT scan, MRI, EEG and screening for infection were perfectly normal, resulting in suspicion of neurological involvement associated with her scleromyxedema. In December 2012 and August 2013, she presented two further episodes of status epilepticus, followed once more by a confusional state, with etiological explorations again proving unfruitful. On this occasion, her confusional state persisted for two months until the initiation of plasmapheresis and IVIG. This combination therapy led to rapid regression of all neurological symptoms and an improvement in her general condition. Discussion The dermato-neuro syndrome is a rare neurological complication of scleromyxedema. Its pathophysiology is unknown. The monoclonal gammopathy induced by the scleromyxedema could account for the patient's hypercoagulable state and for the formation of neutrophilic aggregates leading to impaired microcirculation. Treatment is empirical and poorly codified. The course of the disease is unpredictable and may be lethal.

Background The prognostic relevance of a high blood neutrophil-to-lymphocyte ratio (NLR) has been reported in many cancers, although, to our knowledge, not investigated in patients with Merkel cell carcinoma (MCC) to date. Objective We assessed whether the NLR at baseline was associated with specific survival and recurrence-free survival in MCC. Methods We retrospectively included MCC cases between 1999 and 2015 and collected clinical data, blood cell count at baseline, and outcome. A Cox model was used to identify factors associated with recurrence and death from MCC. Results Among the 75 patients included in the study, a high NLR at baseline (NLR ≥4) was associated with death from MCC in univariate (hazard ratio 2.76, 95% confidence interval 1.15-6.62, P = .023) and multivariate (hazard ratio 3.30, 95% confidence interval 1.21-9.01, P = .020) analysis, but not with recurrence. Limitations Because of the retrospective design, we excluded patients with missing data and not all confounding factors that may influence the NLR were available. Conclusion A high NLR at baseline was independently associated with specific mortality in patients with MCC. The NLR seems to constitute an easily available and inexpensive prognostic biomarker at baseline.

Resume Introduction La chimiotherapie de type mitoguazone, ifosfamide, navelbine, etoposide (MINE) est employee dans la maladie de Hodgkin en rechute ou refractaire aux traitements usuels. Les effets indesirables cutanes de cette chimiotherapie sont rares, comprenant principalement des erythemes et des œdemes des extremites. Plus recemment, quelques cas de panniculite et d’œdemes hyperalgiques ont ete decrits. Observation Nous rapportons le cas d’une jeune fille de 17 ans qui a developpe un œdeme aigu hyperalgique des membres associe a une panniculite du tronc. Ce tableau s’est complete ensuite par l’apparition de bulles et de manifestations inflammatoires des muqueuses, a type de vulvite et de glossite. Les premiers symptomes etaient survenus sept jours apres le debut d’une chimiotherapie de type MINE, sans qu’aucun autre nouveau medicament n’ait ete introduit. L’evolution a ete lentement favorable sous traitement symptomatique a visee antalgique. La chimiotherapie a par la suite ete modifiee. Discussion L’argument d’imputabilite chronologique, l’amelioration spontanee a l’arret du traitement et l’absence d’autre etiologie ont fait evoquer le diagnostic de reaction cutanee secondaire au protocole MINE. La symptomatologie pourrait reposer sur un syndrome de fuite capillaire du a la toxicite de la vinorelbine sur les cellules endotheliales. Ces manifestations secondaires sont a connaitre des dermatologues, notamment l’atteinte inflammatoire des muqueuses, nouvellement decrite.

e21598Background: Nivolumab was the first anti-PD1 therapy to receive a European marketed authorization for the treatment of advanced (unresectable or metastatic) melanoma. To collect and evaluate ...