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Background Eosinophilic annular erythema (EAE) is a rare eosinophil-related skin disease which typically manifests with annular erythematous plaques and severe pruritus. Besides the diagnosis, the treatment of EAE is challenging since relevant published data are sparse. Methods The aim of this study was to assess the underlying diseases, treatments and outcomes of patients with EAE. To this end, we conducted a retrospective multicenter study and a systematic review of the MEDLINE database. Results We included 18 patients with EAE followed in 8 centers. The MEDLINE database search yielded 37 relevant publications reporting 55 cases of EAE with 106 treatment sequences. The most common and efficient treatments included topical or systemic corticosteroids, hydroxychloroquine and dapsone. In refractory patients, a combination of systemic corticosteroids with hydroxychloroquine was associated with 88% of complete clinical response. Discussion To improve the management of EAE patients, we discuss the following treatment strategy: in topical steroid-resistant patients, hydroxychloroquine can be given as first-line systemic treatment. Dapsone, hydroxychloroquine or systemic corticosteroids are second-line options to consider. Last, monoclonal antibodies or JAK inhibitors targeting type 2 inflammation could represent promising last-resort options in refractory patients.

Bullous pemphigoid (BP) is the most common auto immune blistering disease in Europe and its treatment can be challenging. Several published cases reported dupilumab efficiency in refractory patients. We conducted a retrospective multicentric study including 36 patients to evaluate real-life efficiency of dupilumab in BP. Our results suggest that dupilumab in association with high potency topical steroids could be rapidly effective in various clinical forms of BP and seems to be well tolerated in elderly population.

Objective Among specific autoantibodies in DM, the anti–small ubiquitin-like modifier activating enzyme (SAE) antibody is rare. We aim to describe the clinical characteristics, cancer prevalence, and muscle pathology of anti-SAE–positive DM. Methods Patients with a diagnosis of DM and sera positive for the anti-SAE antibody were recruited from 19 centres in this retrospective observational study. The available muscular biopsies were reviewed. We conducted a comparison with anti-SAE–negative DM and a review of the literature. Results Of the patients in the study (n = 49), 84% were women. Skin involvement was typical in 96% of patients, with 10% having calcinosis, 18% ulceration and 12% necrosis; 35% presented with a widespread skin rash. Muscular disease affected 84% of patients, with mild weakness [Medical Research Council (MRC) scale 4 (3, 5)], although 39% of patients had dysphagia. Muscular biopsies showed typical DM lesions. Interstitial lung disease was found in 21% of patients, mainly with organizing pneumonia pattern, and 26% of patients showed dyspnoea. Cancer-associated myositis was diagnosed in 16% of patients and was responsible for the majority of deaths, its prevalence being five times that of the general population. IVIG therapy was administered to 51% of the patients during the course of the disease. Comparison with anti-SAE–negative DM (n = 85) showed less and milder muscle weakness (P = 0.02 and P = 0.006, respectively), lower creatinine kinase levels (P Conclusion Anti-SAE positive DM is a rare subgroup associated with typical skin features but a potentially diffuse rash, a mild myopathy. Interstitial lung disease defines an organizing pneumonia pattern. Cancer associated DM prevalence is five times that of the general population. Trial registration ClinicalTrials.gov, http://clinicaltrials.gov, NCT04637672.

Cutaneous involvement of chronic graft-versus-host disease (cGVHD) has a wide range of manifestations including a lichenoid form with a currently assumed mixed Th1/Th17 signature and a sclerotic form with Th1 signature. Despite substantial heterogeneity of innate and adaptive immune cells recruited to the skin and of the different clinical manifestations, treatment depends mainly on the severity of the skin involvement and relies on systemic, high-dose glucocorticoids alone or in combination with a calcineurin inhibitor. We performed the first study using RNA sequencing to profile and compare the transcriptome of lichen planus cGVHD (n = 8), morphea cGVHD (n = 5), and healthy controls (n = 6). Our findings revealed shared and unique inflammatory pathways to each cGVHD subtype that are both pathogenic and targetable. In particular, the deregulation of IFN signaling pathway was strongly associated with cutaneous cGVHD, whereas the triggering receptor expressed on myeloid cells 1 pathway was found to be specific of lichen planus and likely contributes to its pathogenesis. The results were confirmed at a protein level by performing immunohistochemistry staining and at a transcriptomic level using real-time quantitative polymerase chain reaction.

Idiopathic multicentric Castleman disease (iMCD) is a lymphoproliferative disease of unknown etiology. Deciphering mechanisms involved in CD pathogenesis may help improving patients' care. Six cases of stereotyped sub-diaphragmatic iMCD affecting lower limb-draining areas and associated with severe and often ulcerative lower extremity chronic dermatological condition were identified in our cohort. Pathological examination revealed mixed or plasma-cell type MCD. In three patients, shotgun metagenomics failed to identify any pathogen in involved lymph nodes. Antibiotics had a suspensive effect while rituximab and tocilizumab failed to improve the condition. This novel entity requires a specific approach and exclusion of potentially harmful immunomodulation.

Resume Introduction La plasmocytose cutanee est une dermatose rare decrite en 1976 et observee surtout chez des patients d’ascendance asiatique. Elle forme habituellement des macules et des plaques erythemateuses a brunâtres du tronc et du visage, avec en histologie des infiltrats dermiques composes de plasmocytes matures bien differencies. La pathogenese et la place nosographique de cette affection sont encore mal etablies. Nous presentons un cas de plasmocytose cutanee chez une femme d’ascendance europeenne, particuliere par la presence d’un signe de Darier. Observation Une femme de 56 ans, d’ascendance europeenne, presentait depuis plusieurs annees des macules asymptomatiques brunâtres du dos. Un signe de Darier etait present de facon inconstante. La biopsie cutanee montrait, sur toute la hauteur du derme, des infiltrats moderement denses, pericapillaires ou interstitiels, contenant de nombreux plasmocytes bien differencies. En immunohistochimie, les plasmocytes exprimaient les chaines legeres kappa et lambda sans monotypie ; il n’y avait pas d’expression de l’HHV8 ni de marquage avec l’anticorps anti-treponeme. Biologiquement, il existait une hypergammaglobulinemie polyclonale. La tomodensitometrie thoraco-abdomino-pelvienne etait normale. Le diagnostic de plasmocytose cutanee etait retenu. Une simple surveillance etait instituee dans un premier temps. Discussion La presentation anatomoclinique caracteristique et l’association a une hypergammaglobulinemie polyclonale ont permis d’evoquer le diagnostic de plasmocytose cutanee, bien que cette entite soit exceptionnelle chez les personnes d’ascendance europeenne. Le signe de Darier est parfois observe dans cette affection. Les principaux diagnostics differentiels, infiltrat plasmocytaire d’origine infectieuse et lymphome B de la zone marginale, ont ete ecartes.

The loss of dispersing ability by polycarboxylates ether superplasticizers in alkali-activated slag cements has been widely reported. However, no clear-cut explanation of this phenomenon can be found to date. Therefore, the behaviour of poly(methacrylate-g-poly(ethylene glycol)) superplasticizers in NaOH or Na2CO3 -activated slag pastes was investigated. The observed loss of efficiency of the polymer was not due to a specific property of the slag particles, nor to structural degradation of the polymer in the alkaline solutions. Actually, the ionic strength of the activating solution decreased the solvent quality and changed the polymer conformation, leading to a deterioration of the steric repulsion brought by the side-chains. Moreover, in the Na2CO3 -activated systems, the adsorption behaviour of the polymers was also significantly altered. Here, this was not caused by a low calcium concentration or by a preferential adsorption of the superplasticizer on calcite crystallites. The most plausible explanation was a competitive adsorption with CO32- ions.

No study has assessed the risk factors of progression from discoid lupus erythematosus (DLE) to severe systemic lupus erythematosus (sSLE) (defined as requiring hospitalization and specific treatment).To identify the risks factors of and generate a predicting score for progression to sSLE among patients with isolated DLE or associated with systemic lupus erythematosus with mild biological abnormalities.In this registry-based cohort study, multivariable analysis was performed using risk factors identified from literature and pruned by backward selection to identify relevant variables. The number of points was weighted proportionally to the odds ratio (OR).We included 30 patients with DLE who developed sSLE and 134 patients who did not. In multivariable analysis, among 12 selected variables, an age of25 years at the time of DLE diagnosis (OR, 2.8; 95% CI, 1.1-7.0; 1 point), phototype V to VI (OR, 2.7; 95% CI, 1.1-7.0; 1 point), and antinuclear antibody titers of ≥1:320 (OR, 15; 95% CI, 3.3-67.3; 5 points) were selected to generate the score. Among the 54 patients with a score of 0 at baseline, none progressed to sSLE, whereas a score of ≥6 was associated with a risk of approximately 40%.Retrospective design.In our cohort, an age of25 years at the time of DLE diagnosis, phototype V to VI, and antinuclear antibody titers of ≥1:320 were risk factors for developing sSLE.

Human immunoglobulins are used for treating diverse inflammatory and autoimmune disorders. Eczema is an adverse event reported but poorly described.To describe the clinical presentation, severity, outcome, and therapeutic management of immunoglobulin-associated eczema.This retrospective and descriptive study included a query of the French national pharmacovigilance database, together with a national call for cases among dermatologists.We included 322 patients. Eczema occurred preferentially in men (78.9%) and in patients treated for neurological pathologies (76%). The clinical presentation consisted mainly of dyshidrosis (32.7%) and dry palmoplantar eczema (32.6%); 5% of cases exhibited erythroderma. Sixty-two percent of the eczema flares occurred after the first immunoglobulin course. Eczema was observed with 13 intravenous or subcutaneous immunoglobulin types and recurred in 84% of patients who maintained the same treatment and in 68% who switched the immunoglobulin type. After immunoglobulin discontinuation, 30% of patients still had persistent eczema.Retrospective study, with possible missing data or memory bias.Immunoglobulin-associated eczema occurred with all immunoglobulin types, preferentially in patients with neurologic diseases who required prolonged immunoglobulin treatment. Recurrence was frequent, even after switching the immunoglobulin type, which can lead to a challenging therapeutic situation when immunoglobulin maintenance is required.

Background In leprosy patients, the most commonly reported non-viral co-infections are Tuberculosis, Leishmaniasis, Chromoblastomycosis and Helminths. The presence of a secondary infection is believed to increase the likelihood of leprosy reactions. The purpose of this review was to describe the clinical and epidemiological characteristics of the most reported bacterial, fungal, and parasitic co-infections in leprosy. Methodology/Principal findings Following the PRISMA Extension for Scoping Reviews guidelines, a systematic literature search was conducted by two independent reviewers, resulting in the inclusion of 89 studies. For tuberculosis, a total of 211 cases were identified, with a median age of 36 years and male predominance (82%). Leprosy was the initial infection in 89% of cases, 82% of individuals had multibacillary disease, and 17% developed leprosy reactions. For leishmaniasis, 464 cases were identified, with a median age of 44 years and male predominance (83%). Leprosy was the initial infection in 44% of cases, 76% of individuals presented with multibacillary disease, and 18% developed leprosy reactions. Regarding chromoblastomycosis, we identified 19 cases with a median age of 54 years and male predominance (88%). Leprosy was the primary infection in 66% of cases, 70% of individuals had multibacillary disease, and 35% developed leprosy reactions. Additionally, we found 151 cases of co-infection with leprosy and helminths, with a median age of 43 years and male predominance (68%). Leprosy was the primary infection in 66% of cases, and 76% of individuals presented with multibacillary disease, while the occurrence of leprosy reactions varied from 37% to 81% across studies. Conclusion We observed a male-dominated pattern of co-infections among working-age individuals with multibacillary leprosy. Unlike prior studies reporting increased leprosy reactions in chronic viral co-infections, our findings did not indicate any increase among bacterial, fungal, or parasitic co-infections. Rather, co-infections with tuberculosis and leishmaniasis appeared to reduce leprosy reactions.

Dupilumab is a therapeutic antibody targeting IL-4 and IL-13 receptor subunit alpha used for the treatment of atopic dermatitis (AD) patients. Cases of psoriasis-like reactions induced under dupilumab treatment (DI-Pso) for AD were recently reported. To understand the pathogenesis of DI-Pso, we performed gene expression profiling studies on skin biopsies of DI-Pso (n=7) compared with plaque psoriasis (PSO), AD and healthy controls (HC) (n=4 each). Differential gene expression was performed using enrichment and gene ontology analysis. Gene expression was validated by qPCR and protein levels assessed by immunohistochemistry. Transcriptomic and protein analysis of DI-Pso compared with HC, PSO and AD skins revealed an activation of Th17/IL-23 pathways associated with a significant expression of IL-36, surrogate marker of pustular psoriasis (PP). By contrast, Th2 representative genes' expression were strongly decreased in DI-Pso across comparison. Matching analysis with public data of PP skin corroborated that DI-Pso and PP upstream regulators overlap, greater than with PSO. Furthermore, DI-Pso showed strongly decreased expression of many barrier skin genes compared with HC, PSO and AD. Our data indicate that pathogenesis of DI-Pso relied on a shift of skin immune responses from a Th2 to an IL-36 and Th17 polarization and on intensified skin barrier alterations.

Resume Introduction En metropole, pres de 20 nouveaux cas de lepre sont diagnostiques chaque annee. L’incidence de la tuberculose en France est de 8/100 000 habitants et l’association des 2 mycobacteries a ete exceptionnellement rapportee. Nous rapportons un cas de co-infection lepre Borderline Tuberculoide (BT) et tuberculose disseminee, diagnostique en metropole. Observation Un homme presentait des plaques erythemateuses infiltrees hypoesthesiques diffuses. La biopsie montrait un granulome epithelioide lympho-histiocytaire peri-sudoral et peri-nerveux, avec des bacilles acido-alcoolo-resistants sur la coloration de Ziehl. La PCR Mycobacterium Leprae etait positive, confirmant le diagnostic de lepre, dans une forme BT. Le bilan d’extension revelait un epanchement pleural gauche a predominance lymphocytaire, une adenopathie hilaire droite a centre necrotique sans granulome histologique, une recherche de BK negative, un test quantiFERON-TB™ et une intra-dermo reaction a la tuberculine positifs. Le tableau clinique et radiologique etait en faveur d’une tuberculose disseminee. La quadritherapie anti-tuberculeuse (rifampicine, isoniazide, ethambutol et pyrazinamide) et la clofazimine permettaient une regression des lesions cutanees et extra-cutanees. Discussion Cette rare co-infection associe une lepre souvent lepromateuse evolutive depuis plusieurs annees et une tuberculose souvent pulmonaire d’apparition ulterieure. L’ hypothese physiopathologique est celle de l’immunite croisee (l’immunite anti-tuberculeuse protegeant contre une lepre ulterieure et inversement), etayee par la correlation inverse des deux prevalences et par la protection induite par la vaccination antituberculeuse. Dans la plupart des cas, le traitement antituberculeux et antilepreux permettent une amelioration des deux maladies. Un cas de lepre doit faire rechercher une tuberculose latente afin d’eviter une reactivation, a fortiori si une corticotherapie est associee.

Objectives To describe the clinical and pathological features of biopsy-proven cutaneous vasculitis (CV) associated with SLE, focusing on diagnosis classification and impact on overall SLE activity. Methods Retrospective multicentric cohort study including SLE patients with biopsy-proven CV identified by (i) data from pathology departments of three university hospitals and (ii) a national call for cases. SLE was defined according to 1997 revised ACR and/or 2019 ACR/EULAR criteria. CV diagnosis was confirmed histologically and classified by using the dermatological addendum of the Chapel Hill classification. SLE activity and flare severity at the time of CV diagnosis were assessed independently of vasculitis items with the SELENA-SLEDAI and SELENA-SLEDAI Flare Index. Results Overall, 39 patients were included; 35 (90%) were female. Cutaneous manifestations included mostly palpable purpura (n = 21; 54%) and urticarial lesions (n = 18; 46%); lower limbs were the most common location (n = 33; 85%). Eleven (28%) patients exhibited extracutaneous vasculitis. A higher prevalence of Sjögren’s syndrome (51%) was found compared with SLE patients without CV from the French referral centre group (12%, P Conclusion SLE-related vasculitis seems very rare and other causes of vasculitis should be ruled out before considering this diagnosis. Moreover, in more than half of patients, CV was not associated with another sign of active SLE.

Objectives To describe the effectiveness and safety of biologics for the treatment of relapsing and/or refractory polyarteritis nodosa (PAN). Methods A retrospective European collaborative study was conducted in patients with PAN who received biologics for relapsing and/or refractory disease. Results Forty-two patients with PAN received a total of 53 biologic courses, including TNF-α blockers in 15 cases, rituximab (RTX) in 18 cases, tocilizumab (TCZ) in 10 cases and other biologics in 10 cases. TNF-α blockers and TCZ were mainly used for refractory diseases whereas RTX was mainly initiated for relapsing disease. After a median follow-up of 29 (8–50) months, remission, partial response, treatment failure and treatment discontinuation due to severe adverse events occurred in, respectively, 40%, 13%, 40% and 7% of patients receiving TNF-α blockers, 50%, none, 30% and 20% of TCZ recipients, and 33%, 11%, 56% and none of the RTX recipients. No remission was noted in patients treated with other biologics. Severe adverse events were observed in 14 (28%) patients without significant differences between the three biologics, leading to early biologics discontinuation in only three cases. Conclusion These results suggest that TCZ may be effective in relapsing and/or refractory PAN. Our data warrant further study to confirm these findings.

Dupilumab, an anti-IL-4ɑ monoclonal antibody, has shown a positive benefit-risk ratio when treating moderate to severe atopic dermatitis (AD) in clinical studies (1). High persistence on dupilumab has recently been reported in clinical-practice setting with 77% of patients remaining in treatment for 12 months in a retrospective study including 1,963 patients with AD but reasons for discontinuation were not investigated (2).

Background Paraneoplastic pemphigus (PNP) occurs more often in patients with hematologic malignancies (HMs) than in patients with solid cancer. Lung bronchiolitis obliterans (BO) is a severe complication of PNP. Objective To determine the precise clinical and biologic features of HM-associated PNP and identify factors associated with mortality and survival. Methods Systematic review of previously described cases of PNP associated with HMs. Results A total of 144 patients were included. The HMs were non-Hodgkin lymphoma (52.78%), chronic lymphocytic leukemia (22.92%), Castleman disease (18.60%), and other underlying hematologic malignancy (5.70%). The mortality rate was 57%, and most deaths occurred within the first year after the diagnosis of PNP. Multivariate analysis showed that (1) the presence of antienvoplakin antibodies and BO were significantly associated with death, and (2) a toxic epidermal necrolysis–like clinical pattern, bullous pemphigoid–like clinical pattern, and BO were significantly associated with decreased survival. Limitation Only case reports with sufficient mortality data were included. Conclusion PNP associated with HM has a high mortality rate. The toxic epidermal necrolysis–like and BO-associated forms are independent survival factors in PNP associated with HMs.

Introduction Les vascularites a IgA (VIgA) appartiennent aux vascularites des vaisseaux de petit calibre de la nomenclature de Chapel Hill. Les VIgA de l’adulte sont reputees plus severes que celles de l’enfant et certaines s’associent a des neoplasies solides ou des hemopathies. Les VIgA associees aux gammapathies monoclonales (GM) IgA sont rares et la reponse aux traitements « classiques » peu decrite dans la litterature. Materiel et methodes Nous rapportons les observations de cinq adultes (4H, 1F, âge median 52 ans [35–74]) ayant un purpura vasculaire, une vascularite des vaisseaux de petit calibre, des depots d’IgA en IFD et la presence d’une GM IgA. Les atteintes extra-cutanees etaient : arthralgies (n = 4), douleurs abdominales (n = 2), neuropathie peripherique (n = 2), et plus rarement glomerulonephrite (n = 1), orchite (n = 1)ou serites (n = 1). Tous les patients avaient une GM a IgA, Kappa (3/5), avec un taux Discussion Ces observations illustrent le caractere refractaire des VIgA associees a une GM IgA. L’echec des immunosuppresseurs conventionnels et de la depletion lymphocytaire B temoignent d’un defaut de clairance de la GM ou de ciblage du clone plasmocytaire. Ce concept de vascularite satellite d’une maladie hematologique clonale permet d’enrichir la comprehension de la physiopathologie des VIgA de l’adulte et de les integrer au spectre des GM de signification clinique. Meme si la monotypie des depots cutanes d’IgA n’est pas demontree chez tous ces patients, l’âge de diagnostic de la GM, precoce pour certains, le caractere refractaire de la vascularite, et la RCC et la RCH soutenue apres ciblage du clone sont des arguments plaidant pour un lien causal entre le clone et la vascularite. Ainsi, la realisation d’une immunofixation plasmatique recherchant une GM devrait etre systematique devant une VIgA de l’adulte et repetee en cas de maladie refractaire. Le ciblage du clone doit etre envisage, meme si le taux de la GM est tres faible.

Introduction Des donnees recentes suggerent que pres 50 % des patients atteints de lupus erythemateux cutane (LEC) sont en remission apres 5 ans de traitement. Cependant aucune etude n’a evalue la possibilite de diminuer ou arreter l’hydroxychloroquine (HCQ) apres remission dans le LEC. Materiel et methodes Cette etude retrospective multicentrique a inclus des patients avec LEC chez qui l’HCQ a ete diminuee ou arretee apres remission. Des courbes de Kaplan-Meier ont ete realisees pour evaluer le risque de rechute, definie comme une recidive des lesions justifiant reprise du traitement ou reaugmentation de la dose. Resultats 56 patients ont ete inclus (48 femmes, 87 %) ; l’âge median etait de 46 ans (extremes 11–81) et la duree mediane d’evolution du LEC de 3 ans (extremes 0,04–43). Les types de LEC etaient : discoide (n = 31), lupus engelure (n = 6), panniculite lupique (n = 6), tumidus (n = 19), subaigu (n = 9). Selon les criteres SCLICC, 18 patients (32 %) avaient un lupus systemique (LES), principalement articulaire. Quatre strategies de reduction de l’HCQ ont ete utilisees. La duree mediane avant rechute n’etait pas significativement differente entre ces 4 strategies. Trente-quatre patients (61 %) ont eu au moins une rechute durant le suivi, avec une duree mediane avant rechute > 3,6 ans (0,4–119 mois), incluant 26 patients apres la premiere diminution de dose ou arret de l’HCQ et 8 apres un arret secondaire a une diminution de dose continue ou saisonniere. Au cours du suivi, 39 patients (70 %) ont subi un arret complet de l’HCQ, parmi lesquels 22 ont rechute (56 %). Les lesions acrales etaient le seul facteur significativement associe aux rechutes (HR : 16,74 [95 %CI : 3,38–84,82], p = 0,0006). A l’inverse, il n’y avait pas de difference entre les patients avec ou sans LES (HR 1,01, [95 % CI : 0,41–2,47], p = 0.98). Toutes les rechutes etaient purement cutanees a l’exception d’un patient LES en rechute articulaire. La duree mediane de suivi etait de 5,9 ans (range 0,7–21,3). Au dernier suivi 23 patients (41 %) ne recevaient plus de traitement, 22 (39 %) une dose diminuee d’HCQ et 11 (20 %) avaient repris un traitement actif. Aucun patient de l’etude n’a developpe de maculopathie au cours du suivi. Discussion Cette etude, bien que limitee par son petit echantillon, son caractere retrospectif et l’heterogeneite des strategies utilisees, suggere que reduire ou arreter l’HCQ est un objectif realisable apres remission chez les patients atteints de LEC. La toxicite maculaire de l’HCQ au long cours est bien connue ; la diminution ou l’arret de l’HCQ pourrait prevenir cet effet secondaire severe.

Introduction La dermatite atopique (DA) est une dermatose inflammatoire chronique frequente bien connue chez l’enfant; sa prevalence est de l’ordre de 10 a 25 %. En France, selon Objectifs Peau [SFD 2017] ce sont 2,5 millions de francais de 15 ans et plus qui seraient atteints de DA [4,65 %]. Chez l’adulte, les presentations cliniques peuvent etre trompeuses et variables au fil du temps chez un meme individu. Materiel et methodes Etude multicentrique transversale en vie reelle, sans modification de prise en charge dont l’objectif etait de decrire les caracteristiques cliniques et epidemiologiques de la DA de l’adulte. Les patients adultes atteints de DA etaient inclus consecutivement a l’issue d’une consultation spontanee. Les donnees etaient rapportees par le dermatologue en charge du sujet. Resultats Nous presentons les resultats issus des donnees obtenues entre decembre 2018 et mars 2020. 30 dermatologues hospitaliers et liberaux francais avaient inclus 816 patients. Il y avait plus de femmes [55,4%]. L’âge moyen au moment de l’inclusion etait de 36,9 ans. 25,1 % des patients avaient debute leur DA a l’âge adulte. On notait un debut precoce avant l’âge de 2 ans chez 45,1 %. Une evolution biphasique (debut dans l’enfance, remission complete et reapparition a l’âge adulte) etait rapportee chez 32,8 % des patients. Les comorbidites atopiques associees etaient la rhinite saisonniere (48,1 %), l’asthme (43,2 %), la conjonctivite allergique (33,2 %) et les allergies alimentaires (20,1 %). On notait des troubles anxieux et syndrome depressifs chez respectivement 16,4 % et 10,1 % des patients. 30,3 % des patients avaient eu recours aux medecines alternatives et 48,9% avaient consulte un allergologue a l’âge adulte. La severite de la maladie a l’inclusion etait elevee (IGA moyen a 2,7). La repartition des formes phenotypique etait la suivante : forme clinique dite « classique » (zones bastions atteintes, sans localisations predominantes) chez 44,6% des individus suivie de la forme « tete et cou » (20,1 %) ; viennent ensuite par ordre de frequence l’eczema chronique des mains (13,3 %), l’eczema nummulaire (8,2 %), l’erythrodermie (4,5 %), le prurigo (4,2 %), la dyshidrose (4,1 %) et l’eczema craquele (1,1 %). Discussion Les resultats de l’etude DAPHNE montrent l’heterogeneite de la dermatite atopique de l’adulte avec des phenotypes et evolution variables. Contrairement aux donnees precedentes de la litterature trouvant environ 12% d’evolution biphasique de la DA, nous notons plus de formes recidivantes. Les formes d’apparition tardive sont moins frequentes (25 % versus 20/40 %). Les formes classiques, « tete et cou » et eczema chronique des mains sont les plus representees. La relative severite de la DA au moment de l’inclusion peut etre lie a un biais de recrutement plus hospitalier que liberal. Il s’agit de la premiere etude en vie reelle de description de la dermatite atopique de l’adulte en France fait par des dermatologues.

Introduction La lepre est une des premieres causes de neuropathies dans le monde. Son evolution peut etre marquee par des complications immunologiques appelees reactions lepreuses. La reaction de type 1 ou reaction de reversion (RR) est responsable d’une infiltration rapide et douloureuse des lesions cutanees et de nevrites potentiellement severes et irreversibles. La reaction de type 2 ou erytheme noueux lepreux (ENL), est une reaction a complexes immuns caracterisee par des nodules inflammatoires douloureux associes a des signes generaux. La prise en charge therapeutique des reactions lepreuses repose avant tout sur la corticotherapie generale prolongee, source de morbidite importante. La thalidomide, alternative therapeutique efficace dans l’ENL, est d’utilisation limitee par sa toxicite et sa tolerance mediocre. Au cours de la RR, des immunosuppresseurs conventionnels comme la ciclosporine ou l’azathioprine ont ete utilises mais les donnees d’efficacite et de tolerance sont parcellaires dans cette indication, et leur disponibilite reduite dans les pays d’endemie. Dans la litterature, une vingtaine de cas d’efficacite du MTX ont ete rapportes. L’objectif de l’etude vise a analyser l’efficacite et la tolerance du methotrexate (MTX) au cours des reactions lepreuses ainsi que l’epargne cortisonique. Materiel et methodes Il s’agit d’une etude retrospective multicentrique incluant l’ensemble des patients suivis pour une lepre et ayant recu du MTX dans le cadre d’une reaction lepreuse de type 1 et 2 depuis 2016. Resultats Un total de 13 patients ont ete inclus, 5 femmes et 8 hommes. Les reactions etaient : ENL (n = 10) et RR (n = 9). La dose mediane de MTX etait de 20 mg par semaine avec une duree mediane de traitement de 14 mois. Au cours du traitement, 9/13 patients n’ont pas eu de recidive reactionnelle sous MTX. Parmi les 4 patients ayant recidive, on notait 3 RR et 2 ENL. La dose de prednisone etait reduite de 86 % en moyenne lors de l’arret du MTX. Un sevrage en corticoides etait obtenu chez 5 des 11 patients sous CTC. La tolerance du MTX etait correcte. Un patient a presente une cytolyse grade II. Parmi les 8 patients ayant arrete le MTX (3 pour conception, 1 pour cytolyse, 1 pour nausees, 2 pour inefficacite, 1 pour COVID-19) 6 ont recidive dans un delai median de 8 mois apres l’arret du MTX (3–13). Discussion Il s’agit de la plus grande cohorte rapportant l’interet du MTX pour limiter les recidives reactionnelles au cours de la lepre et permettre une epargne cortisonique. Le MTX semble etre une alternative de choix dans le traitement des reactions lepreuses corticodependantes avec un profil de tolerance favorable. L’efficacite suspensive plaide en faveur d’une utilisation prolongee pour limiter la frequence des rechutes a l’arret. La disponibilite et le cout reduit du MTX permettent d’envisager une utilisation dans les pays d’endemie.

Introduction L’erytheme annulaire a eosinophiles (EAE) est une dermatose eosinophilique qui se manifeste typiquement par des plaques annulaires et erythemateuses et un prurit severe. Certaines formes ont ete decrites comme satellite de cancers solides. Du fait de la rarete de la pathologie, et du peu de donnees disponibles dans la litterature, le traitement est mal codifie et peut s’averer tres complexe devant certaines formes refractaires. Materiel et methodes Le but de cette etude etait de colliger les pathologies associees, les traitements et leur efficacite dans l’objectif de proposer une meilleure prise en charge therapeutique dans l’EAE. Ainsi, nous avons conduit une etude retrospective multicentrique et une revue systematique de la litterature avec la base MEDLINE. Pour etre inclus les patients devaient avoir un diagnostic clinique et histologique. La reponse therapeutique etait classifiee comme remission complete (RC), partielle (PR) ou absence de reponse. Resultats Notre etude retrospective et notre revue systematique ont permis d’inclure respectivement 18 et 55 patients, pour un total de 131 sequences therapeutiques analysees. Les traitements les plus frequemment retrouves et les plus efficaces etaient les corticoides locaux, les corticoides systemiques, l’hydroxychloroquine et la dapsone, avec respectivement 62, 41, 57 et 46 % de RC avec ces traitements. Chez les patients refractaires, une combinaison de corticoides systemiques avec de l’hydroxychloroquine etait associee a une RC dans 88 % des cas. Les resultats complets sont presentes dans la. Discussion Nos resultats permettent de discuter les traitements suivants: chez les patients resistants aux corticoides locaux, l’hydroxychloroquine peut etre propose en premiere ligne de traitement systemique. La dapsone ou l’association hydroxychloroquine et corticoides systemiques sont des options de seconde ligne, a considerer chez les patients refractaires. Enfin, les anticorps monoclonaux ou les inhibiteurs de JAK ciblant l’inflammation de type Th2 semblent etre des options interessantes chez les patients avec EAE refractaires aux traitements conventionnels.

Background Hypocomplementemic urticarial vasculitis (HUV) is a rare systemic vasculitis. We aimed to describe the kidney involvement of HUV in a multicenter national cohort with an extended follow-up. Methods All patients with HUV (international Schwartz criteria) with a biopsy-proven kidney involvement, identified through a survey of the French Vasculitis Study Group (FVSG), were included. A systematic literature review on kidney involvement of HUV was performed. Results Twelve patients were included, among whom 8 had positive anti-C1q antibodies. All presented with proteinuria, from mild to nephrotic, and 8 displayed acute kidney injury (AKI), requiring temporary haemodialysis in 2. Kidney biopsy showed membrano-proliferative glomerulonephritis (MPGN) in 8 patients, pauci-immune crescentic GN or necrotizing vasculitis in 3 patients (with a mild to severe interstitial inflammation), and an isolated interstitial nephritis in 1 patient. C1q deposits were observed in the glomeruli (n = 6), tubules (n = 4) or renal arterioles (n = 3) of 8 patients. All patients received corticosteroids, and 9 were also treated with immunosuppressants or apheresis. After a mean follow-up of 8.9 years, 6 patients had a preserved renal function, but 2 patients had developed stage 3–4 chronic kidney disease (CKD) and 4 patients had reached end-stage kidney disease (ESKD), among whom 1 had received a kidney transplant. Conclusion Renal involvement of HUV can be responsible for severe AKI, CKD and ESRD. It is not always associated with circulating anti-C1q antibodies. Kidney biopsy shows mostly MPGN or crescentic GN, with frequent C1q deposits in the glomeruli, tubules or arterioles.

Background Cutaneous polyarteritis nodosa is a form of medium-sized vessel vasculitis. Despite a disabling and prolonged course, data on treatment efficacy and safety remain scarce. Objectives We aimed to describe treatment efficacy and safety in patients with cutaneous polyarteritis nodosa. Methods This multicenter retrospective, observational study, recorded clinical and biologic data together with treatments received. The primary outcome was the rate of complete response at month 3. Secondary outcomes assessed drug survival and safety. Results We included 68 patients who received a median of 2 therapeutic lines (interquartile range, 1-3). Overall, complete response was achieved in 13 of 42 (31%) patients with colchicine, 4 of 17 (23%) with dapsone, 11 of 25 (44%) with glucocorticoids (GCs) alone, 1 of 9 (11%) with nonsteroidal anti-inflammatory drugs, 11 of 13 (84%) with GCs+azathioprine, and 7 of 15 (47%) with GCs+methotrexate. GCs+azathioprine had the best drug survival (median duration, 29.5 months; interquartile range, 19.5-36.0). Response at month 3 was decreased with peripheral neurologic involvement (odds ratio, 0.19; 95% confidence interval, 0.03-0.81; P = .04). Overall, the rate of treatment-related adverse events was 18%, which led to the discontinuation of treatment in 7% of patients. Limitation Retrospective study. Conclusion Colchicine seems to confer good benefit-risk balance in cutaneous polyarteritis nodosa without peripheral sensory neuropathy. GCs+azathioprine seem the best treatment in the event of relapse.

Background: Glucocorticoids (GCs) plus rituximab (RTX) represent the first-line treatment of nonviral mixed cryoglobulinemia vasculitis (CryoVas). However, data on therapeutic management and outcome of patients refractory to RTX are lacking. Methods: We conducted a European collaborative retrospective multicenter study of patients with nonviral mixed CryoVas refractory to RTX and performed a literature review. Results: Twenty-six original cases and 7 additional patients from the literature were included. All patients but one had type 2 cryoglobulinemia, and causes were autoimmune disease (51%), malignant hemopathy (12%) or essential CryoVas (42%). CryoVas was primary refractory to RTX in 42%, while 58% had an initial response to RTX before immune escape. After RTX failure, patients received a median of 1 (IQR, 1–3) line of treatment, representing 65 treatment periods during follow-up. Main treatments used were GCs in 92%, alkylating agents in 43%, RTX in combination with other treatments in 46%, and belimumab in 17%. Combination of anti-CD20 plus belimumab, alkylating agents alone and anti-CD20 plus alkylating agents provided the highest rates of clinical response in 100% 82% and 73%, respectively, but showed poor immunological response, in 50%, 30% and 38%, respectively. Rates of severe infection were 57%, 9% and 0% in patients receiving anti-CD20 plus belimumab, alkylating agents alone and anti-CD20 plus alkylating agents, respectively. Conclusion: In patients with nonviral mixed CryoVas refractory to RTX, anti-CD20 plus belimumab, and alkylating agents associated or not with anti-CD20, provide the highest rates of clinical response. However, anti-CD20 plus belimumab was frequently associated with severe infections.

Introduction Les inhibiteurs de checkpoint immunitaire (ICI), tels que les anticorps anti-PD-1, PD-L1 et anti-CTLA-4, sont efficaces dans de nombreux cancers. Cependant, entre 70 et 90 % des patients presentent des effets indesirables (EI). Le lupus erythemateux immuno-induit (LEII) est un EI rare. L’objectif de notre etude etait d’estimer la prevalence des LEII dans une population traitee en oncodermatologie pour un melanome metastatique et decrire les caracteristiques cliniques et biologiques des patients presentant un LEII. Materiels et methodes Nous avons interroge retrospectivement la base de donnees Melbase afin d’estimer la prevalence de LEII dans la population de melanome traitee par ICI dans notre centre et la base nationale de pharmacovigilance (BNPV). Resultats Sur 330 patients traites par ICI pour un melanome dans notre centre depuis 2013, 2 patients presentaient un LEII (0,6 %). Dans la BNPV, 11 LEII etaient enregistres, dont 6 femmes. L’âge median etait de 65 ans [42-82], les cancers primitifs etaient des adenocarcinomes pulmonaires (5), des melanomes (4), un cancer du sein et un cancer ovarien. Les traitements recus etaient : nivolumab en monotherapie (4) ou associe a ipilimumab (1), pembrolizumab (3), atezolizumab (2) durvalumab (1). Le delai median entre l’introduction des ICI et les premiers symptomes etait de 3 mois [0,5-6]. Huit patients sur 11 presentaient un lupus cutane isole confirme histologiquement, 6 etaient des femmes, 1 avait des lesions muqueuses, 5 avaient des anticorps anti-SSA. Les traitements utilises etaient des dermocorticoides (8/8), du plaquenil (3/8), des corticoides oraux (2/8) permettant une evolution favorable. L’ICI etait poursuivi ou repris chez 3 patients sans recidive. Trois patients sur 11 presentaient un lupus erythemateux systemique (LES) repondant aux criteres EULAR/ACR 2019, 2 patients presentaient une atteinte articulaire avec des anticorps anti-ADN natifs, d’evolution favorable sous corticoides et plaquenil, un presentait des serites et un syndrome d’Evans traite par corticoides et immunoglobulines intraveineuses. Chez ces 3 patients, l’immunotherapie a ete interrompue sans reprise. Discussion Les lupus induits sont des EI rares dont les inducteurs les plus incrimines sont l’hydralazine et la procainamide. Les LEII sont peu rapportes et de diagnostic difficile de par l’absence de criteres diagnostiques etablis et la presentation clinique et biologique variee. Son incidence est estimee a 0,48 % dans l’etude de Michot et al. [1] , proche de celle de notre experience (0,6 %). L’atteinte cutanee isolee semble plus frequente que la presentation systemique avec une predominance de forme subaigue et d’anti SSA. Les traitements classiques du lupus sont a proposer et l’arret de l’immunotherapie est a discuter selon la presentation clinique. Conclusion Le LEII est un EI rare mais parfois severe. Le diagnostic peut etre difficile devant des signes cliniques et biologiques varies.

Myelodysplastic syndromes (MDS) are malignant hematopoietic stem cell disorders characterized by dysplastic and ineffective blood cell production leading to blood cytopenias, and a risk of transformation to acute myeloid leukemia (AML). Skin lesions in patients with MDS include Sweet syndrome (SS), leucocytoclastic vasculitis, leukemia cutis and histiocytoid SS (H-SS), among others,.1,2 H-SS is a histological variant of SS differing from classical neutrophilic SS by a dermal infiltrate mostly made of immature cells of the myeloid lineage, that has been associated with myeloid malignancies.

International audience; Scleromyxedema is a rare skin and systemic mucinosis that is usually associated with monoclonal gammopathy (MG). In this French, multicenter, retrospective study of 33 patients, we investigated the clinical and therapeutic features of MG-associated scleromyxedema. Skin molecular signatures were analyzed using a transcriptomic approach. Skin symptoms included papular eruptions (100%), sclerodermoid features (91%), and leonine facies (39%). MG involved an IgG isotype in all patients, with a predominant λ light chain (73%). Associated hematologic malignancies were diagnosed in 4/33 patients (12%) (smoldering myeloma, n=2; chronic lymphoid leukemia, n=1; and refractory cytopenia with multilineage dysplasia n=1). Carpal tunnel syndrome (33%), arthralgia (25%) and dermato-neuro syndrome (DNS) (18%) were the most common systemic complications. One patient with mucinous cardiopathy died of acute heart failure. Intravenous immunoglobulin (HDIVig) treatment alone or in combination with steroids appeared to be quite effective in nonsevere cases (clinical complete response achieved in 13/31 patients). Plasma cell-directed therapies using lenalidomide and/or bortezomib with dexamethasone and HDIVig led to a significant improvement in severe cases (HDIVig-refractory or cases with central nervous system or cardiac involvement). The emergency treatment of DNS with combined plasmapheresis, HDIVig, and high-dose corticosteroids induced the complete remission of neurological symptoms in 4/5 patients. Quantitative reverse transcriptase-PCR (RT-PCR) analysis of 6 scleromyxedema skin samples showed significantly higher profibrotic pathway levels (transforming growth factor β (TGFβ) and collagen-1) than in healthy skin. Prospective studies targeting plasma cell clones and/or fibrotic pathways are warranted for long-term scleromyxedema management.

Heavy-chain deposition disease (HCDD) is characterized by tissue deposits of a truncated monoclonal immunoglobulin heavy-chain (HC) on basement membranes. Diagnosis is usually made on kidney biopsy, showing nodular glomerulosclerosis with HC deposits which can be missed, resulting in delay in diagnosis. We report four γ1-HCDD patients presenting with cutis laxa, hypocomplementemia and hypoalbuminemia. In two patients, unsuspected HCDD was revealed by cutis laxa and diagnosis was made on skin biopsy. In all patients, serum albumin and complement represented surrogate markers for disease monitoring. In γ-HCDD, extrarenal manifestations such as cutis laxa may precede renal injury and are precious tools for an early diagnosis, which is crucial to avoid progression of irreversible renal and elastic tissue damage.

Hypocomplementemic urticarial vasculitis (HUV), called anti-C1q vasculitis in the 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides, is a rare systemic vasculitis of unknown etiology, affecting small vessels. HUV is characterized by urticarial lesions, hypocomplementemia and systemic manifestations, mostly musculoskeletal and ocular, but also gastrointestinal, pulmonary and kidney involvement. Anti-C1q antibodies are detected in only half of the patients, and low C1q seems to represent a more sensitive marker. Published data on the therapeutic management are scarce in the literature. Hydroxychloroquine and colchicine seem to represent effective first-line therapies. In patient with relapsing or refractory disease, response rates appeared slightly higher for corticosteroids together with conventional immunosuppressive agents, in particular azathioprine, mycophenolate mofetil, or cyclophosphamide, while a rituximab-based regimen tended to have higher efficacy. The best strategy for treating HUV has yet to be defined.

Urticarial vasculitis (UV) is rare systemic disease characterised by a dermal capillary inflammation responsible of long-lasting urticarial lesions, and sometimes some systemic manifestations.1 2 UV can be separated into two different entities: hypocomplementemic UV (HUV) and normocomplementemic UV (NUV). Treatment of UV depends on the severity of the disease and the occurrence of relapses. In patients refractory to colchicine or dapsone, glucocorticoids (GCs), conventional immunosuppressive agents or rituximab (RTX) can be used,1 2 leading to significant morbidity. Interleukin-1β (IL-1β) inhibitors are biologics mainly used in autoinflammatory diseases.3 We report here a French retrospective multicentr study of patients treated with IL-1β inhibitors for persistent disease despite GCs and/or immunosuppressive agents. This French retrospective multicentre study was conducted in four departments of Dermatology and Internal Medicine. We included patients with persistent UV to at …