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BACKGROUND: Adverse pregnancy outcomes (APO) occur in 35% of patients with pemphigoid gestationis (PG). No biological predictor of APO has been established yet. OBJECTIVES: To assess a potential relationship between the occurrence of APO and the serum value of anti-BP180 antibodies at the time of PG diagnosis. METHODS: Multicentre retrospective study conducted from January 2009 to December 2019 in 35 secondary and tertiary care centres. INCLUSION CRITERIA: (i) diagnosis of PG according to clinical, histological and immunological criteria, (ii) ELISA measurement of anti-BP180 IgG antibodies determined at the time of PG diagnosis with the same commercial kit and (iii) obstetrical data available. RESULTS: Of the 95 patients with PG included, 42 had one or more APO, which mainly corresponded to preterm birth (n = 26), intrauterine growth restriction (IUGR) (n = 18) and small weight for gestational age at birth (n = 16). From a ROC curve, we identified a threshold of 150 IU ELISA value as the most discriminating to differentiate between patients with or without IUGR, with 78% sensitivity, 55% specificity, 30% positive and 91% negative predictive value. The threshold >150 IU was confirmed using a cross-validation based on bootstrap resampling, which showed that the median threshold was 159 IU. Upon adjusting for oral corticosteroid intake and main clinical predictors of APO, an ELISA value of >150 IU was associated with the occurrence of IUGR (OR = 5.11; 95% CI: 1.48-22.30; p = 0.016) but not with any other APO. The combination of blisters and ELISA values higher than 150 IU led to a 2.4-fold higher risk of all-cause APO (OR: 10.90; 95% CI: 2.33-82.3) relative to patients with blisters but lower values of anti-BP180 antibodies (OR of 4.54; 95% CI 0.92-34.2). CONCLUSION: These findings suggest that anti-BP180 antibody ELISA value in combination with clinical markers is helpful in managing the risk of APO, in particular IUGR, in patients with PG.

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OBJECTIVE: To assess the role of the anti-TIF1γ auto-antibody (aAb) IgG2 isotype as a biomarker of cancer in anti-TIF1γ aAb-positive adult DM. METHODS: International multicentre retrospective study with the following inclusion criteria: (i) diagnosis of DM according to ENMC criteria; (ii) presence of anti-TIF1γ IgG aAb determined using an in-house addressable laser bead immunoassay (ALBIA) from cryopreserved serums sampled at time of DM diagnosis and (iii) available baseline characteristics and follow-up data until the occurrence of cancer and/or a minimum follow-up of 1 year for patients without known cancer at diagnosis. Detection and quantification of anti-TIF1γ IgG2 aAb was done using the in-house ALBIA. In addition, a recent ELISA commercial kit was used for anti-TIF1γ IgG aAb quantification. RESULTS: A total of 132 patients (mean age 55±15 years) of whom 72 (54.5%) had an associated cancer were analysed. The association between the presence of cancer and the presence of anti-TIF1γ IgG2 aAb was statistically significant (P = 0.026), with an OR of 2.26 (95% CI: 1.10, 4.76). Patients with cancer displayed significantly higher anti-TIF1γ IgG2 aAb ALBIA values with a median value of 1.15 AU/ml (IQR: 0.14-9.76) compared with 0.50 AU/ml (IQR: 0.14-1.46) for patients without cancer (P = 0.042). In addition, patients with cancer displayed significantly higher anti-TIF1γ IgG aAb ELISA values with a median value of 127.5 AU/ml (IQR: 81.5-139.6) compared with 93.0 AU/ml (IQR: 54.0-132.9) for patients without cancer (P = 0.004). CONCLUSION: These results suggest considering anti-TIF1γ IgG2 ALBIA and IgG ELISA values as biomarkers of cancer in anti-TIF1 γ aAb-positive adult DM.

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OBJECTIVE: To deep sequence the TRIM33 gene in tumours from patients with cancer-associated anti-TIF1γ autoantibody-positive dermatomyositis (DM) as TRIM33 somatic mutations in tumours may trigger this auto-immune disease. METHODS: Next generation sequencing of tumour DNA samples from patients with cancer-associated anti-TIF1γ autoantibody-positive DM. Fourteen tumours from 13 anti-TIF1γ autoantibody-positive DM individuals were sequenced along with two control tumours from non-DM individuals. RESULTS: Fourteen probable somatic variants from four tumours were identified in the TRIM33 gene. CONCLUSION: These results are in accordance with the previous report of Pinal-Fernandez et al. and support the hypothesis of a role of TRIM33 gene mutations in the pathophysiology of anti-TIF1γ autoantibody-positive DM.

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OBJECTIVE: 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. METHODS: 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. RESULTS: 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). CONCLUSION: 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.

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BACKGROUND: Lymphomatoid papulosis (LyP) is classified as an indolent cutaneous lymphoma, but outcome dramatically worsens if LyP is associated with lymphoma. The frequency of this association remains unclear in the literature. Here, we assess the frequency and risk factors of association between LyP and another lymphoma in an 11-year retrospective study conducted in 8 dermatology departments belonging to the French Study Group on Cutaneous Lymphoma (FSGCL). PATIENTS AND METHODS: Patients with LyP were identified and data extracted from the FSGCL registry between 1991 and 2006. Patients were followed up to January 2014. Age, sex, number of skin lesions, histologic subtype, and genotype were recorded at baseline. Risk factors were determined using univariate and multivariate analysis. Cumulative probability of association was calculated using the Kaplan-Meier method. RESULTS: We observed 52 cases of lymphomas (cutaneous, n = 38; systemic, n = 14) in 44 of 106 patients (41%). Lymphoma diagnosis was concomitant with or prior to LyP diagnosis in 31 cases and occurred during the course of LyP in 21 cases (cutaneous, n = 14; systemic, n = 7; median delay: 5 years; interquartile range: 1.5-7 years). In multivariate analysis, main prognostic factors for association between LyP and another lymphoma were older age (odds ratio [OR]: 1.05 per year; 95% confidence interval [CI]: 1.01-1.08; p = .011) and presence of a T-cell clone in LyP lesions (OR: 7.55; 95% CI: 2.18-26.18; p = .001). CONCLUSION: Older age and presence of a T-cell clone in LyP lesions are risk factors for associated lymphomas in patients with LyP. These findings should help to identify patients who require close management in clinical practice. IMPLICATIONS FOR PRACTICE: The management of lymphomatoid papulosis (LyP) is that of an indolent cutaneous lymphoma, based on its excellent prognosis. However, this good prognosis is altered if LyP is associated with lymphoma. Furthermore, risk factors for and frequency of this association remain unclear in the literature. The results presented here demonstrate a high rate of association between LyP and other lymphomas (41%) as well as a long median delay of occurrence (5 years), which emphasizes the need for prolonged follow-up of patients with LyP. Moreover, two main risk factors (i.e., older age and presence of a T-cell clone in LyP lesions) are highlighted, which should help clinical practitioners to identify patients who require close management.

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OBJECTIVE: To assess high-risk human papillomavirus (HR HPV) cervical infections and their type distribution among healthy women in Guadeloupe, French West Indies. METHODS: The details of consecutive non-pregnant women who attended cervical cancer screening and had HPV genotyping performed at the largest pathology laboratory on the island from January 1, 2013 to December 31, 2014 were recorded retrospectively. All women with available HPV genotyping results were included in the study. RESULTS: HR HPV genotyping results for 618 women (median age 42 years) were collected. The overall prevalence rate of HR HPV cervical infection was 36.1% (95% confidence interval (CI) 32.3-40.0%), with the following type distribution: HPV 16 or 18 irrespective of other HPV types, 7.3% (95% CI 5.4-9.6%); other HR HPV types excluding HPV 16 or 18, 28.8% (95% CI 25.3-32.5%). The prevalence rates of overall HR HPV and HR HPV other than 16 or 18 infection increased significantly (p<0.001) with the severity of cytology grade, from 19.7% for normal cytology to 53.8% in atypical squamous cells of undetermined significance (ASC-US) and 67.7% in low-grade squamous intraepithelial lesions (LSIL). CONCLUSION: The high prevalence rate of HR HPV cervical infection with genotypes other than 16 and 18 in Guadeloupe, irrespective of age and the cytology grade, suggests a potential benefit of the new nine-valent HPV vaccine to prevent HPV infection-related cancers in this Caribbean country.

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BACKGROUND: There are no reliable epidemiological data on sarcoidosis in the French West Indies, although this disease is known to be more frequent and more severe in Black African-Americans and West Indians. OBJECTIVES: This retrospective study aimed to assess the incidence and prevalence of sarcoidosis in Guadeloupe over a 7-year period and to determine its epidemiological, clinical, and evolutionary characteristics. METHODS: Patients were identified through the computerized databases of the three pathology laboratories and two hospitals on the islands of Guadeloupe. Histologically proven cases of sarcoidosis were selected. All patients were recalled at a single study time-point. RESULTS: A total of 75 patients were identified. These included 44 women and 31 men (sex ratio: 1.4), with a mean ± standard deviation (SD) age of 47 ± 14 years and Fitzpatrick skin types IV-VI. The average incidence was 2.28 per 100,000 inhabitants per year (95% confidence interval [CI] 1.69-3.02). The prevalence of sarcoidosis in 2009 was 21.09 per 100,000 inhabitants (95% CI 16.00-26.18). Most patients (61/71, 85.9%) exhibited multiple organ involvement; the mean ± SD number of organs involved was 2.6 ± 1.1. The initiation of systemic therapy was required in 75.7% of cases. Several lines of treatment were necessary in 41.5% of affected patients. At the study time-point, seven patients were found to have died. Four of these deaths were directly attributable to sarcoidosis (mortality rate: 5.3%). CONCLUSIONS: This epidemiological study on sarcoidosis in Guadeloupe reveals a low incidence of the disease and a high degree of severity as evidenced by the average number of affected organs, the high frequency of extrathoracic organ involvement, the frequent use of corticosteroids, and a mortality rate of 5.3%.

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BACKGROUND: There are no reported epidemiological data regarding autoimmune pemphigus in the Afro-Caribbean population. OBJECTIVES: To present the epidemiology of autoimmune pemphigus on the island of Guadeloupe (French West Indies, 400,736 inhabitants, mostly black Caribbean of African European descent). MATERIALS AND METHODS: Five-year prospective study. Inclusion of the incident cases when directly referred to the Dermatology Department or secondarily referred by their private practice dermatologist once identified by the computerized databases of the Guadeloupian pathology laboratories. RESULTS: World-population-standardized incidence was 6.96 (95% CI: 3.41-10.52) for pemphigus vulgaris and 3.75 (95% CI: 1.12-6.39) for pemphigus foliaceus. Patients usually live in the rural countryside, whereas 75% of the population of Guadeloupe Island live in an urban environment. CONCLUSION: We report a high incidence of autoimmune pemphigus in Guadeloupe, especially for the foliaceus type, and the existence of particular epidemiological features such as the rural countryside habitat.

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