La maladie de Kimura (étude rétrospective multicentrique de 25 cas et relation avec la maladie associée aux IgG4)

La maladie de Kimura (MK) est une maladie lymphoproliférative d'étiologie inconnue, décrite principalement en Asie. Les données dans les pays occidentaux sont rares, surtout en ce qui concerne les modalités thérapeutiques. Une relation avec la maladie associée aux IgG4 (IgG4-RD) a été évoquée. Nous avons analysé les données cliniques, thérapeutiques et histopathologiques chez 25 patients français avec une MK.Les patients étaient majoritairement des hommes (84%), d un âge médian de 42 ans (16-89), avec un suivi médian de 3,5 ans (0,7-18). Des nodules cutanés étaient présents chez 88% des patients. Une atteinte des ganglions lymphatiques, des glandes lacrymales ou salivaires et du rein était rapportée respectivement dans 45%, 24% et 12% des cas. On observait de nombreuses similitudes clinico-pathologiques avec les 27 cas publiés d atteinte cutanée d IgG4-RD. L hybridation in situ par sonde EBER était négative dans 17/18 cas (94%). En cas de lésion résécable, l exérèse chirurgicale était le traitement de première intention avec 100% de réponse complète et 60% de rechutes. Dans les cas inopérables, à lésions multiples, ou avec atteinte extra-cutanée, la corticothérapie générale permettait une rémission complète ou partielle dans 100% des cas avec 50% de rechute. Le thalidomide, la ciclosporine, ou l'interféron-alpha étaient de bonnes alternatives aux corticoïdes généraux avec un taux de réponse de 100%, mais avec respectivement 100%, 20% et 50% de rechutes.PARIS6-Bibl.Pitié-Salpêtrie (751132101) / SudocSudocFranceF

An atypical persistent eruption of adult-onset Still's disease with neutrophilic urticarial dermatosis-like dermal features: A case report and review of the literature

Adult‐onset Still's disease (AOSD) is a rare systemic inflammatory disorder with unknown etiology. Cutaneous manifestations of AOSD could be typical (evanescent maculopapular rash, salmon‐pink, and non‐ or mildly pruriginous) or atypical (persistent lesions with pruritus). In typical AOSD, microscopic assessment shows non‐specific features such as very mild dermal inflammatory infiltrate (lymphocytes and neutrophils), and in atypical AOSD, it shows highly suggestive dyskeratotic/necrotic keratinocytes with neutrophilic and lymphocytic dermal infiltrate.1-3 Neutrophilic urticarial dermatosis (NUD) is an autoinflammatory condition recently described as recurrent urticarial eruption vanishing within 24 hours with histologically marked neutrophilic infiltrate and leukocytoclasia. NUD is a rare cutaneous presentation of AOSD.4 We report two cases of atypical persistent eruption of AOSD with dyskeratotic/necrotic keratinocytes and in one case leukocytoclasia (as an NUD‐like dermal pattern) that has rarely been described previously. Knowledge of the clinico‐pathological spectrum of atypical AOSD should facilitate diagnosis of this rare and severe disease

An atypical persistent eruption of adult-onset Still's disease with neutrophilic urticarial dermatosis-like dermal features: A case report and review of the literature

Adult‐onset Still's disease (AOSD) is a rare systemic inflammatory disorder with unknown etiology. Cutaneous manifestations of AOSD could be typical (evanescent maculopapular rash, salmon‐pink, and non‐ or mildly pruriginous) or atypical (persistent lesions with pruritus). In typical AOSD, microscopic assessment shows non‐specific features such as very mild dermal inflammatory infiltrate (lymphocytes and neutrophils), and in atypical AOSD, it shows highly suggestive dyskeratotic/necrotic keratinocytes with neutrophilic and lymphocytic dermal infiltrate.1-3 Neutrophilic urticarial dermatosis (NUD) is an autoinflammatory condition recently described as recurrent urticarial eruption vanishing within 24 hours with histologically marked neutrophilic infiltrate and leukocytoclasia. NUD is a rare cutaneous presentation of AOSD.4 We report two cases of atypical persistent eruption of AOSD with dyskeratotic/necrotic keratinocytes and in one case leukocytoclasia (as an NUD‐like dermal pattern) that has rarely been described previously. Knowledge of the clinico‐pathological spectrum of atypical AOSD should facilitate diagnosis of this rare and severe disease

Next generation sequencing for personalized therapy: About a class III BRAF N581K mutation associated to NRAS Q61L mutation in malignant melanoma: Case report

In metastatic stage, therapeutic approach for malignant melanoma is particularly based on performance status, metastatic sites, and BRAF V600 status (BRAF V600E/V600K or V600R (class I BRAF mutations). In most cases, BRAF mutations and NRAS mutations are mutually exclusive to each other. However, some rare BRAF mutations class III are preferentially associated with a NRAS mutation, leading to the MAP Kinase pathway activation and subsequent cell proliferation. Melanomas with this double mutation are rare and difficult to treat because of the lack of codified therapeutic options. We report a patient with metastatic melanoma, harboring class III BRAF mutation (N581K) associated to NRAS mutation (Q61L) with treatment failure. He was treated in second line, after immunotherapy, by monotherapy of MEK inhibitor (MEKi), which underline the interest of NGS (Next Generation Sequencing) to early identify all mutations and enabling onco-dermatologist to discuss a treatment. Rare BRAF non V600 mutations represent 3 to 14% of melanoma mutants and the aim of this communication is to promote the next generation sequencing to extend the paradigm of individually therapeutic approach with target therapy into different spectrum of melanoma patients

Real-Life Study of the Benefit of Concomitant Radiotherapy with Cemiplimab in Advanced Cutaneous Squamous Cell Carcinoma (cSCC): A Retrospective Cohort Study

Background: Cemiplimab is a monoclonal antibody targeting the PD-1, and phase II trials have shown its efficacy in the treatment of advanced cutaneous squamous cell carcinoma in patients who are not candidates for curative surgery or radiation therapy as a first- or later-line treatment. A synergistic antitumoral response has been demonstrated with concurrent radiotherapy and PD1-immunotherapy. However, no real-life study has demonstrated this effect in advanced cutaneous squamous cell carcinoma. Methods: We conducted a real-life retrospective cohort study to investigate the benefit of concomitant therapy in 33 patients treated with cemiplimab at the University Hospital of Caen, alone (C group) or concomitant to radiotherapy (C/RT group). Our primary objectives were to evaluate the best overall response and objective response rate. Our secondary objectives were the disease control rate, median time to response, progression-free survival, overall survival, clinical benefit of radiotherapy, and safety data. After stopping cemiplimab administration, we performed a follow-up of our patients and performed a descriptive study. Results: We reported an objective response rate of 45.5%, including 47.6% in the cemiplimab group versus 41.6% in the concomitant group. The addition of radiotherapy to cemiplimab enables an earlier clinico-radiological response, with a median duration of 5.5 months in the cemiplimab group versus 3 months in the concomitant therapy group. The response to treatment was prolonged despite discontinuation of cemiplimab, with 91.6% (n = 11/12) and 83.3% (n = 10/12) patients in complete or partial remission at 6 months and 1 year after cessation of cemiplimab and no switch to another oncological treatment, respectively. Radiation therapy also provided a therapeutic effect in 83.3% of the patients in the concomitant group, without increasing the occurrence of adverse events. Conclusions: Our study confirms the efficacy of cemiplimab and radiotherapy in the management of advanced cutaneous squamous cell carcinoma. Concomitant therapy permitted to obtain an earlier radiological response, a beneficial local therapeutic effect of radiotherapy, without any safety alert

18F-FDG PET/CT versus Diagnostic Contrast-Enhanced CT for Follow-Up of Stage IV Melanoma Patients Treated by Immune Checkpoint Inhibitors: Frequency and Management of Discordances over a 3-Year Period in a University Hospital

Aim: To perform a comprehensive analysis of discordances between contrast-enhanced CT (ceCT) and 18F-FDG PET/CT in the evaluation of the extra-cerebral treatment monitoring in patients with stage IV melanoma. Materials and methods: We conducted a retrospective monocentric observational study over a 3-year period in patients referred for 18F-FDG PET/CT and ceCT in the framework of therapy monitoring of immune checkpoint (ICIs) as of January 2017. Imaging reports were analyzed by two physicians in consensus. The anatomical site responsible for discordances, as well as induced changes in treatment were noted. Results: Eighty patients were included and 195 pairs of scans analyzed. Overall, discordances occurred in 65 cases (33%). Eighty percent of the discordances (52/65) were due to 18F-FDG PET/CT scans upstaging the patient. Amongst these discordances, 17/52 (33%) led to change in patient’s management, the most frequent being radiotherapy of a progressing site. ceCT represented 13/65 (20%) of discordances and induced changes in patients’ management in 2/13 cases (15%). The most frequent anatomical site involved was subcutaneous for 18F-FDG PET/CT findings and lung or liver for ceCT. Conclusions: Treatment monitoring with 18F-FDG PET/CT is more efficient than ceCT and has a greater impact in patient’s management

Evaluation of Thalidomide Treatment of Patients With Chronic Erythema Multiforme

International audienc

Efficacité rapide de l'anifrolumab dans le lupus érythémateux cutané réfractaire : une étude prospective de 11 patient atteints de lupus érythémateux systémiques

for the EMSED (Etude des maladies systémiques en dermatologie) study group.International audienceNo abstract availabl

Localization, Morphologic Features, and Chemical Composition of Calciphylaxis-Related Skin Deposits in Patients With Calcific Uremic Arteriolopathy

International audienceImportance: Calcific uremic arteriolopathy (CUA), a rare, potentially fatal, disease with calcium deposits in skin, mostly affects patients with end-stage renal disease who are receiving dialysis. Chemical composition and structure of CUA calcifications have been poorly described.Objectives: To describe the localization and morphologic features and determine the precise chemical composition of CUA-related calcium deposits in skin, and identify any mortality-associated factors.Design, Setting, and Participants: A retrospective, multicenter cohort study was conducted at 7 French hospitals including consecutive adults diagnosed with CUA between January 1, 2006, and January 1, 2017, confirmed according to Hayashi clinical and histologic criteria. Patients with normal renal function were excluded. For comparison, 5 skin samples from patients with arteriolosclerosis and 5 others from the negative margins of skin-carcinoma resection specimens were also analyzed.Main Outcomes and Measures: Localization and morphologic features of the CUA-related cutaneous calcium deposits were assessed with optical microscopy and field-emission-scanning electron microscopy, and the chemical compositions of those deposits were evaluated with μ Fourier transform infrared spectroscopy, Raman spectroscopy, and energy dispersive radiographs.Results: Thirty-six patients (median [range] age, 64 [33-89] years; 26 [72%] female) were included, and 29 cutaneous biopsies were analyzed. Calcific uremic arteriolopathy and arteriolosclerosis skin calcifications were composed of pure calcium-phosphate apatite. Calcific uremic arteriolopathy vascular calcifications were always circumferential, found in small to medium-sized vessels, with interstitial deposits in 22 (76%) of the samples. A thrombosis, most often in noncalcified capillary lumens in the superficial dermis, was seen in 5 samples from patients with CUA. Except for calcium deposits, the vessel structure of patients with CUA appeared normal, unlike thickened arteriolosclerotic vessel walls. Twelve (33%) patients died of CUA.Conclusions and Relevance: Calcific uremic arteriolopathy-related skin calcifications were exclusively composed of pure calcium-phosphate apatite, localized circumferentially in small to medium-sized vessels and often associated with interstitial deposits, suggesting its pathogenesis differs from that of arteriolosclerosis. Although the chemical compositions of CUA and arteriolosclerosis calcifications were similar, the vessels' appearances and deposit localizations differed, suggesting different pathogenetic mechanisms