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Chronic urticaria is a common skin disorder with heterogeneous causes. In the absence of physical triggers, chronic urticarial rash is called idiopathic or spontaneous. The objective of this study was to identify the molecular and cellular bases of a disease condition displayed by two unrelated patients aged over 60 years who presented for two decades with a chronic urticaria resistant to standard therapy that occurred in the context of systemic inflammation not triggered by cold. In both patients, a targeted sequencing approach using a next generation technology identified somatic mosaic mutations in NLRP3, a gene encoding a key inflammasome component. The study of several of both patients' cell types showed that, despite the late onset of the disease, NLRP3 mutations were not found to be restricted to myelomonocytic cells. Rather, the data obtained strongly suggested that the mutational event occurred very early, during embryonic development. As shown by functional studies, the identified mutations-an in-frame deletion and a recurrent NLRP3 missense mutation-have a gain-of-function effect on NLRP3-inflammasome activation. Consistently, a complete remission was obtained in both patients with anti-IL-1 receptor antagonists. This study unveils that in late-onset chronic urticaria, the search for autoinflammatory markers and somatic mosaic NLRP3 mutations may have important diagnostic and therapeutic consequences.

To provide an epidemiologic description of Kikuchi-Fujimoto disease (KFD), and to describe its relationship with systemic lupus erythematosus (SLE) in a population of sub-Saharan origin.Patients were retrospectively included on the basis of lymph node histology compatible with KFD reported in Martinique from 1991 until 2013. In order to describe the characteristics of the disease in a larger cohort, we subsequently included more patients of Afro-Caribbean origin from Guadeloupe and French Guiana.In Martinique, mean annual incidence between 1991 and 2013 was 2.78 cases for 1 million inhabitants (95% confidence interval 1.73-3.93). A total of 36 Afro-Caribbean patients from the 3 French American regions were included. Mean age was 30.5 years (range 5-59 years) and the female:male ratio was 3:1. The main characteristics were cervical adenopathies (88.8%), fever (83.3%), asthenia (73.0%), weight loss (64.4%), and recurrence in 33.3%. KFD was associated with lupus (n = 9 for SLE, n = 2 for cutaneous lupus) in 36.6% (11 of 30).We report the first epidemiologic description of KFD in a population of sub-Saharan origin. According to our data, this disease is present in the black African diaspora and is strongly associated with autoimmune diseases, particularly lupus.

To describe chronic chikungunya manifestations seen during the outbreak in the Caribbean from December 2013 to January 2015.Patients were seen at our center, the only rheumatology department in Martinique Island, and were examined by a senior rheumatologist using a standard care report form. Chikungunya was diagnosed collectively based on consensus among all clinicians. The median time from onset of acute chikungunya to the first rheumatology consultation was calculated, severity was evaluated based on clinical scales and the degree of joint destruction, and each patient's treatment was recorded.For the 147 patients analyzed, the median time between onset of acute chikungunya and the first rheumatology consultation was 8 months. After review of each patient's medical record, 19 (12.9%) were diagnosed as having epidemic-influenced chikungunya. Four distinct rheumatologic patterns were observed in the remaining patients (those with compatible history and positive serologic findings): 47 patients (32%) had reactivation of painful chronic mechanical manifestations, 9 patients (6.1%) had fibromyalgia, 45 patients (30.6%) met criteria for spondyloarthritis (as evaluated before the chikungunya virus infection in all patients) and experienced a flare, and 27 patients (18.4%), with no history of joint disease, developed de novo bilateral symmetric chronic inflammatory joint disease in response to chikungunya virus infection. For inflammatory arthritis, most patients were treated with methotrexate (up to 25 mg/week), with good response and tolerance. Thirteen patients were treated with conventional doses of anti-tumor necrosis factor agents, with good tolerance and efficacy as expected.The term "chronic chikungunya syndrome" covers multiple etiologies. Compliance with the French Society of Rheumatology recommendations, careful recording of patient histories, and serologic verification help prevent errors inherent to the epidemic context and ensure early therapeutic intervention for these patients. To avoid late initiation of treatment, patients should receive rheumatologic consultation as early as possible.

International audience; Background: Guillain-Barré syndrome (GBS) has been reported to be associated with Zika virus (ZIKV) infection in case reports and retrospective studies, mostly on the basis of serological tests, with the problematic cross-reacting antibodies of the Flavivirus genus. Some GBS cases do not exhibit a high level of diagnostic certainty. This prospective study aimed to describe the clinical profiles and the frequency of GBS associated with ZIKV during the ZIKV outbreak in Martinique in 2016.Methods: We recorded prospective data from GBS meeting levels 1 or 2 of diagnostic certainty for the Brighton Collaboration, with proof of recent ZIKV infection and negative screening for etiologies of GBS.Results: Of the sample of 34 patients with suspected GBS during the outbreak, 30 had a proven presence of GBS, and 23 had a recent ZIKV infection. The estimated GBS incidence rate ratio (2016 vs 2006-2015) was 4.52 (95% confidence interval, 2.80-7.64; P = .0001). Recent ZIKV infection was confirmed by urine reverse-transcription polymerase chain reaction (RT-PCR) analysis in 17 cases and by serology in 6 cases. Patients, 65% of whom were male, had a median age of 61 years (interquartile range, 56-71 years) and experienced severe GBS. Electrophysiological tests were consistent with the primary demyelinating form of the disease.Conclusions: ZIKV infection is usually benign, when symptomatic, but in countries at risk of ZIKV epidemics, adequate intensive care bed capacity is required for management of severe GBS cases. Arbovirus RNA detection by RT-PCR should be part of the management of GBS cases.