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Incidence and Mortality of Bullous Pemphigoid in France
A major increase in the incidence of BP has been recently reported in the United Kingdom. In addition, there are some controversies about the over-mortality of BP patients. The primary objective was to reevaluate the incidence of BP in France as compared with that we estimated 15 years ago. The secondary objective was to assess mortality of BP patients. BP incidence was retrospectively estimated from all BP cases diagnosed between January 2000 and December 2005 in three French regions with a total population of 3.858 million inhabitants. BP mortality was assessed from a prospective cohort accrued during the same time period. A total of 502 incident BP patients (mean age: 82.6±8.8years) were identified. Overall estimated incidence was 21.7 cases per million persons per year (95%CI:19.8–23.7 cases per million persons per year), which is about 3-fold higher than the incidence that we estimated 15 years ago. In the population aged 70 years or above, BP incidence was 162 cases per million per year (95%CI:147–177 cases per million per year). The overall 1-year survival rate was 62% (95% CI: 56–67%). The risk of death for BP patients was more than six times greater than that for the general population (SMR:6.60; 95%CI:5.47–7.90). The incidence of BP in France has increased 3-fold in the last 15 years. BP is associated with high mortality.
Journal Article
The role of gender in the perception of autism symptom severity and future behavioral development
Background
Increasing attention is being paid to the higher prevalence of boys with Autism Spectrum Disorder (ASD) and to the implications of this ratio discrepancy on our understanding of autism in girls. One recent avenue of research has focused on caregiver’s concern, suggesting that autism might present differently in boys and girls. One unexplored factor related to concerns on child development is whether socio-cultural factors such as gender-related expectations influence the evaluation of symptom severity and predictions about future behavioral development.
Methods
The latter concerns were the focus of the present study and were explored by investigating laypeople’s judgment of the severity of autism symptoms using an online parent role-playing paradigm, in which participants were asked to rate vignettes depicting the behaviors of a child in different everyday life scenarios. The child’s gender and the severity of ASD symptoms were manipulated to examine the effect of gender on the perception of symptom severity.
Results
Results suggest that there are no gender differences in perceived symptom severity and associated degree of concern for 5-year-old boys and girls but that there is a gender difference in perceived future atypicality at 15 years old, with boys being rated as more likely to be perceived as atypical by their peers at that age than girls.
Conclusions
Investigating parent’s cognition about their child’s future behavioral development can provide additional information regarding delayed diagnosis of autistic girls.
Journal Article
Bullous Pemphigoid: A Review of its Diagnosis, Associations and Treatment
Bullous pemphigoid (BP) is the most common autoimmune subepidermal blistering disease in Western countries, and typically affects the elderly. BP is immunologically characterized by tissue-bound and circulating autoantibodies directed against either the BP antigen 180 (BP180, or BPAG2) or the BP antigen 230 (BP230, or BPAG1e), or even both, which are components of hemidesmosomes involved in the dermal–epidermal cohesion. Risk factors for BP include old age, neurologic diseases (dementia, Parkinson’s disease, cerebrovascular disease), and some particular drugs, including loop diuretics, spironolactone and neuroleptics. The spectrum of clinical presentations is extremely broad. Clinically, BP is an intensely pruritic erythematous eruption with widespread blister formation. In the early stages, or in atypical, non-bullous variants of the disease, only excoriated, eczematous or urticarial lesions (either localized or generalized) are present. The diagnosis of BP relies on immunopathologic findings, especially based on both direct and indirect immunofluorescence microscopy observations, as well as on anti-BP180/BP230 enzyme-linked immunosorbent assays (ELISAs). BP is usually a chronic disease, with spontaneous exacerbations and remissions, which may be accompanied by significant morbidity. In the past decade, potent topical corticosteroids have emerged as an effective and safe first-line treatment for BP, but their long-term feasibility is still controversial. Newer therapeutic agents targeting molecules involved in the inflammatory cascade associated with BP represent future alternatives to classical immunosuppressant drugs for maintenance therapy.
Journal Article
Why Is Sexualization Dehumanizing? The Effects of Posture Suggestiveness and Revealing Clothing on Dehumanization
Research has shown that sexualized people are perceived as possessing fewer traits of a human being. Most scholars have argued that these effects are driven by revealing clothing, with targets wearing swimsuits or lingerie being perceived as possessing less mind and less humanness in comparison with nonsexualized targets. However, revealing clothing in these studies was often confounded with other sexualizing factors, such as posture suggestiveness, and, so, the aspects which lead people to perceive women in object-like ways remain unclear. This article begins to fill this gap by examining the role of two key sexualizing factors, namely revealing clothing and posture suggestiveness, on objectification-related traits. After exposure to a picture of a woman, 223 participants were asked to indicate the extent to which this woman possessed warmth, competence, and morality. For competence and warmth, we found an interaction between revealing clothing and posture suggestiveness: Posture suggestiveness caused less attribution of warmth and competence to women wearing revealing clothing, but not for women wearing less revealing clothing. For morality, we found that women in suggestive (vs. nonsuggestive) postures were perceived as possessing less morality, regardless of the type of clothing. The implications of these findings for the field are discussed.
Journal Article
Eosinophil Cationic Protein (ECP), a predictive marker of bullous pemphigoid severity and outcome
Bullous Pemphigoid (BP) is an inflammatory rare autoimmune bullous dermatosis, which outcome cannot be predicted through clinical investigations. Eosinophils are the main immune infiltrated cells in BP. However, the release of Major Basic Protein (MBP), Eosinophil Derived Neurotoxin (EDN), and Eosinophil Cationic Protein (ECP) upon eosinophil activation has still not been evaluated with respect to BP development. MBP, EDN and ECP were measured by ELISA in serum (n = 61) and blister fluid (n = 20) of patients with BP at baseline, and in serum after 2 months of treatment (n = 41). Eosinophil activation in BP patients was illustrated at baseline by significantly higher MBP, EDN and ECP serum concentrations as compared with control subjects (n = 20), but without distinction according to disease severity or outcome. EDN and ECP values were even higher in the blister fluids (
P
< 0.01 and
P
< 0.05, respectively), whereas MBP values were lower (
P
< 0.001). ECP serum concentration decreased after 60 days of treatment in BP patients with ongoing remission but not in patients who later relapsed (
P
< 0.05). A reduction of at least 12.8 ng/mL in ECP concentrations provided a positive predictive value for remission of 81%, showing that ECP serum variation could be a useful biomarker stratifying BP patients at risk of relapse.
Journal Article
Innate Immune Cell–Produced IL-17 Sustains Inflammation in Bullous Pemphigoid
Bullous pemphigoid (BP) is an autoimmune skin disease characterized by the binding of autoantibodies to components of the hemidesmosome structure, resulting in an inflammatory response and subepidermal blister formation. To investigate the role of immune orientation in the inflammatory processes associated with disease progression, blister fluid, serum, and biopsy specimens were collected from 31 consecutive BP patients. Blister fluids displayed high levels of IL-6, IL-17, IL-22, and IL-23, whereas transforming growth factor-β was increased in BP sera. However, neither immunocytochemistry on a trans-differentiation model of IL-17-producing peripheral blood mononuclear cells nor immunohistochemistry on BP biopsy specimens could demonstrate the presence of T helper type 17 lymphocytes. Instead, innate immune cells, especially neutrophils, produced IL-17 at the skin lesional site. Of note, superpotent topical corticosteroid application quickly and markedly reduced both IL-17 expression and clinical signs of BP. Consistently, IL-17 upregulated matrix-metalloprotease-9 and neutrophil elastase expression, two proteases involved in blister formation, thereof further demonstrating its role in the progress of BP. Finally, IL-17-induced matrix degradation, originated from neutrophil activation, initiated the formation of an amplification loop of the inflammatory response that could represent the underlying phenomenon leading to the maintenance and even disease extent. Thus, our results could open new therapeutic strategies for BP patients.
Journal Article
A Single Cycle of Rituximab for the Treatment of Severe Pemphigus
In this study of 21 patients with severe pemphigus whose disease was refractory to or dependent on systemic corticosteroids or who had contraindications to corticosteroids, 18 patients (86%) had a complete remission after a single cycle of rituximab treatment. Two patients had serious infections, one of which resulted in death. The efficacy of rituximab for pemphigus must be weighed against the risk of severe adverse events.
In this study of 21 patients with severe pemphigus, 18 patients (86%) had a complete remission after a single cycle of rituximab treatment. The efficacy of rituximab must be weighed against the risk of severe adverse events.
Pemphigus is a life-threatening autoimmune blistering disease affecting the skin and mucosa. It is mediated by pathogenic autoantibodies directed against desmoglein 1 and desmoglein 3, adhesion molecules of the epidermis that are responsible for the cohesion between keratinocytes in skin and mucosa, respectively.
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Patients with severe pemphigus require long-term treatment with corticosteroids and other immunosuppressive drugs, which can lead to serious adverse events.
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Rituximab, a monoclonal antibody directed against the CD20 antigen of B lymphocytes, has been demonstrated to be effective in various autoimmune diseases
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and in occasional cases of life-threatening pemphigus.
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Recently, the combination of . . .
Journal Article