Background Skin psoriasis precedes the onset of psoriatic arthritis (PsA) in 84% of patients with psoriasis. Dermatologists have an important role to screen psoriasis patients for PsA. The efficiency ...of PsA screening remains unknown. Objective We sought to determine the point prevalence of undiagnosed PsA in patients with psoriasis using a systematic search of the literature and meta-analysis. Methods PubMed, Cochrane, and Embase database searches yielded 394 studies for review. No study aimed to determine the prevalence of undiagnosed PsA in patients with psoriasis. We assumed that the prevalence of newly diagnosed PsA in patients with psoriasis at the time they seek medical care could be a sound estimate of this value. Seven epidemiological studies and 5 studies on PsA screening questionnaires allowed us to clearly identify patients with newly diagnosed PsA and were selected for review. Results The prevalence of undiagnosed PsA was 15.5% when all studies were considered and 10.1% when only epidemiological studies were considered. Limitations Data were obtained from studies not designed to address the question at hand. Heterogeneity was high ( I2 = 96.86%), and therefore a random effects model was used. Conclusion The high prevalence of undiagnosed PsA in patients with psoriasis adds to the recommendation that dermatologists need to screen all patients with psoriasis for PsA.
Our scientific knowledge of bullous pemphigoid (BP) has dramatically progressed in recent years. However, despite the availability of various therapeutic options for the treatment of inflammatory ...diseases, only a few multicenter controlled trials have helped to define effective therapies in BP. A major obstacle in sharing multicenter-based evidences for therapeutic efforts is the lack of generally accepted definitions for the clinical evaluation of patients with BP. Common terms and end points of BP are needed so that experts in the field can accurately measure and assess disease extent, activity, severity, and therapeutic response, and thus facilitate and advance clinical trials. These recommendations from the International Pemphigoid Committee represent 2 years of collaborative efforts to attain mutually acceptable common definitions for BP and proposes a disease extent score, the BP Disease Area Index. These items should assist in the development of consistent reporting of outcomes in future BP reports and studies.
Mucous membrane pemphigoid encompasses a group of autoimmune bullous diseases with a similar phenotype characterized by subepithelial blisters, erosions, and scarring of mucous membranes, skin, or ...both. Although knowledge about autoimmune bullous disease is increasing, there is often a lack of clear definitions of disease, outcome measures, and therapeutic end points. With clearer definitions and outcome measures, it is possible to directly compare the results and data from various studies using meta-analyses. This consensus statement provides accurate and reproducible definitions for disease extent, activity, outcome measures, end points, and therapeutic response for mucous membrane pemphigoid and proposes a disease extent score, the Mucous Membrane Pemphigoid Disease Area Index.
BACKGROUND Thalidomide use in cutaneous sarcoidosis is based on data from small case series or case reports. The objective of this study was to evaluate the efficacy and safety of thalidomide in ...severe cutaneous sarcoidosis. METHODS This study consisted of a randomized, double-bind, parallel, placebo-controlled, investigator-masked, multicenter trial lasting 3 months and an open-label study from month 3 to month 6. Adults with a clinical and histologic diagnosis of cutaneous sarcoidosis were included in nine hospital centers in France. Patients were randomized 1:1 to oral thalidomide (100 mg once daily) or to a matching oral placebo for 3 months. In the course of an open-label follow-up from month 3 to month 6, all patients received thalidomide, 100 mg to 200 mg daily. The proportions of patients with a partial or complete cutaneous response at month 3, based on at least a 50% improvement in three target lesions scored for area and infiltration, were compared across randomization groups. RESULTS The intent-to-treat population included 39 patients. None of them had a complete cutaneous response. Four out of 20 patients in the thalidomide group (20%) vs four out of 19 patients in the placebo group (21%) had a partial cutaneous response at month 3 (difference in proportion of −1% 95% CI, −26% to +24% for thalidomide vs placebo, P = 1.0). Eight patients with side effects were recorded in the thalidomide group vs three in the placebo group. We observed a large number of adverse event-related discontinuations in patients taking thalidomide in the first 3 months (four patients with thalidomide, zero with placebo) and in the 3 following months (five patients). CONCLUSIONS At a dose of 100 mg daily for 3 months, our results do not encourage thalidomide use in cutaneous sarcoidosis. TRIAL REGISTRY ClinicalTrials.gov ; No.: NCT0030552; URL: www.clinicaltrials.gov
Background A rare variant of mycosis fungoides (MF), syringotropic MF (STMF) is characterized by a particular tropism of the lymphocytic infiltrate for the eccrine structures, and included in the ...follicular subtype of MF in the World Health Organization–European Organization for Research and Treatment of Cancer classification of cutaneous lymphomas. Objective We sought to determine the clinicopathologic features and disease course of patients with STMF. Methods A retrospective study was conducted to identify patients with STMF from 1998 to 2013. Results Nineteen patients were included: 15 men and 4 women, mean age 55 years (range, 24-86). Most had multiple lesions (n = 16, 84%) with associated alopecia (n = 12, 63%) and/or punctuated aspect (n = 12, 63%). Palms or soles were involved in 10 cases (53%). Folliculotropism was found in 13 cases (68%). After a median follow-up of 70 months (range, 2-140), 3 patients died, 1 from disease-related death. The 5-year overall and disease-specific survival were 100%. The disease-specific survival was significantly higher than in 54 patients with folliculotropic MF without syringotropism (5-year disease-specific survival, 74%; 95% confidence interval, 58%-94%, P = .02). Limitations Retrospective setting is a limitation. Conclusions In the spectrum of adnexotropic MF, STMF appears as a distinct entity from follicular MF, with peculiar clinical characteristics and natural history.
Background Occupational exposure is reported as playing a substantial causative role in systemic sclerosis (SSc). Objective We sought to compare the characteristics of SSc in patients with and ...without occupational exposure to crystalline silica/solvents. Methods In all, 142 patients with SSc were enrolled in this prospective study. An expert committee performed blind evaluation of occupational exposure to crystalline silica/solvents. Results Patients exposed to crystalline silica more often exhibited: diffuse cutaneous SSc ( P = .02), digital ulcers ( P = .05), interstitial lung disease ( P = .0004), myocardial dysfunction ( P = .006), and cancer ( P = .06). Patients exposed to solvents more frequently developed: diffuse cutaneous SSc ( P = .001), digital ulcers ( P = .01), interstitial lung disease ( P = .02), myocardial dysfunction ( P = .04), and cancer ( P = .003); in addition, these patients were more frequently anti-Scl 70 positive and anticentromere negative. Under multivariate analysis, significant factors for SSc associated with exposure to silica/solvents were: male gender (odds ratio 19.31, 95% confidence interval 15.34-69.86), cancer (odds ratio 5.97, 95% confidence interval 1.55-23.01), and digital ulcers (odds ratio 2.42, 95% confidence interval 1.05-5.56). Limitations The cohort originated from a single geographic region. Conclusion Occupational exposure to crystalline silica/solvents is correlated with more severe forms of SSc characterized by: diffuse cutaneous involvement, interstitial lung disease, general microangiopathy (digital ulcers and myocardial dysfunction), and association with cancer. Occupational exposure should be systematically checked in all patients with SSc, as exposed patients seem to develop more severe forms of SSc.
Incidence and risk factors for striae gravidarum Picard, Damien, MD, PhD; Sellier, Séverine, MD; Houivet, Estelle, PhD ...
Journal of the American Academy of Dermatology,
10/2015, Volume:
73, Issue:
4
Journal Article
Background Few studies have evaluated pemphigus treatments according to the definitions of the consensus statement. Prognostic factors for complete remission off therapy (CRoffT) remain unknown. ...Objective We sought to assess the rate of CRoffT in patients with pemphigus treated with different regimens. Methods In all, 134 patients with pemphigus were included in a retrospective, multicenter study. Primary end point was the rate of CRoffT. Prognostic factors for CRoffT were determined using univariate and multivariate analyses. Results Eighty patients with pemphigus vulgaris, 47 with pemphigus foliaceus, and 7 with paraneoplastic pemphigus were included. Mean age was 60 ± 18 years. Patients were treated either with medium (≤0.5 mg/kg/d) (n = 32) or high (≥1 mg/kg/d) (n = 59) doses of prednisone, or without systemic corticosteroids (n = 43). Mean follow-up was 77 ± 64 months. In all, 68 patients (50.7%) achieved CRoffT (95% confidence interval 42.3%-59.2%) after a mean treatment duration of 36 ± 39 months, including 47 of 80 patients with pemphigus vulgaris (58.7%) and 21 of 47 with pemphigus foliaceus (44.7%). Main prognostic factors for CRoffT were initial mucosal involvement (hazard ratio 2.2; 95% confidence interval 1.05-4.58; P = .036) and younger age (<61 years) (hazard ratio 2.5; 95% confidence interval 1.18-5.12; P = .0167). The rate of long-lasting CRoffT was 44%, with a mean follow-up after treatment withdrawal of 59 ± 50 months. Limitations This was a retrospective study. Conclusion The rate of CRoffT was 51%. Patients with pemphigus vulgaris were more likely to achieve CRoffT than those with pemphigus foliaceus.
Background Previous reports show that parathyroid hormone (PTH) concentrations may vary widely depending on the assay used to assess PTH. In this cross-sectional study, we aim to determine the ...usefulness of standardizing blood handling for optimal interpretation of PTH in patients with chronic kidney disease. Study Design Diagnostic test study. Setting & Participants Predialysis blood was sampled in 34 long-term hemodialysis patients at a single academic medical center. Index Test PTH was measured by using 6 different automated second-generation assays (Elecsys, Advia Centaur, LIAISON, Immulite, Architect, and Access assays), 3 blood specimen types (serum, EDTA plasma, and citrate plasma), and 2 consecutive days of measurement (after thawing and 18 hours later with samples having been let at room temperature). Reference Test None. Results A mixed statistical analysis model showed that the nature of the assay ( P < 0.001) and nature of the blood sample ( P < 0.001) significantly influenced variability in PTH concentrations, whereas day of measurement (day 1 or 2) did not ( P = 0.5). Most PTH variability was caused by observations (96.8%), then manufacturer's kit (2.5%), and last, specimen type (0.7%). PTH concentrations measured in citrate plasma were lower with every assay method used than those observed in serum or EDTA plasma. The interaction between manufacturer and specimen type was of moderate statistical significance ( P = 0.04). To evaluate the potential clinical consequence of PTH measure variability, we classified patients according to Kidney Disease Outcomes Quality Initiative cutoff values (PTH < 150 pg/mL; PTH, 150 to 300 pg/mL; and PTH > 300 pg/mL). Overall, statistical classification agreement was moderate to high for comparison between assays and high to very high between different blood samples and between days of measurement. However, we found that up to 11 of 34 patients were classified in different categories with some assays (LIAISON versus Architect) and up to 7 of 34 in different categories with different blood specimen type (citrate versus plasma in LIAISON assay). Limitations This is a cross-sectional study that used single lots of reagents. There currently is no reference method for the measurement of PTH and no recombinant PTH standard for PTH assay. Conclusion PTH variability caused by the nature of the assay and/or blood specimen type is large enough to potentially influence clinical decision making. A specified collection method therefore should be used for PTH measurements. In routine practice, we recommend serum PTH over EDTA or citrate plasma.
Our scientific knowledge of pemphigus has dramatically progressed in recent years. However, despite the availability of various therapeutic options for the treatment of inflammatory diseases, only a ...few multicenter controlled trials have helped to define effective therapies in pemphigus. A major obstacle in comparing therapeutic outcomes between centers is the lack of generally accepted definitions and measurements for the clinical evaluation of patients with pemphigus. Common terms and end points of pemphigus are needed so that experts in the field can accurately measure and assess disease extent, activity, severity, and therapeutic response, and thus facilitate and advance clinical trials. This consensus statement from the International Pemphigus Committee represents 2 years of collaborative efforts to attain mutually acceptable common definitions for pemphigus. These should assist in development of consistent reporting of outcomes in future studies.
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