HLA-E*0101/0103X is associated with susceptibility to pemphigus vulgaris: a case-control study

Pemphigus vulgaris (PV) is a life-threatening, autoimmune blistering disease of the skin and mucous membranes. The relationship between PV and human leukocyte antigen (HLA) has been studied in several reports. Previous reports have demonstrated that HLA-E polymorphisms may have a role in the susceptibility to various autoimmune diseases. Our aim was to evaluate the role of HLA-E gene polymorphisms in the pathogenesis of PV in a Turkish population. A total of 49 patients with PV and 50 healthy subjects were enrolled into the study. We sequenced and analyzed the HLA-E gene from genomic DNA obtained from peripheral blood samples of the study groups. HLA-E haplotyping was performed by Sanger sequencing of PCR products of the HLA-E gene and HLA-E alleles determined by using SeqScape® software according to the World Health Organization (WHO) Nomenclature Committee for Factors of the HLA System. The frequency of the HLA-E*0101/*0103X genotype in male patients with PV was found to be significantly higher than in men in the control group (P=0.023). In addition, the frequency of the HLA-E*0103X/*0103X genotype was significantly lower in patients with PV than the control group (P=0.040). We also detected that the frequency of the HLA-E*0101/*0103X genotype in patients with mucocutaneous type PV and the frequency of the HLA-E*0101/*0101 genotype in patients with mucosal type PV was significantly higher than those in other types of PV (P=0.001 and P=0.006). The results of this study indicate that carrying the HLA-E*0101/0103X genotype may increase the risk of PV in male patients.  </p

Retrospective analysis of methylprednisolone treatment alone and in combination with methotrexate in patients with extensive alopecia areata

Alopecia areata (AA) is a chronic autoimmune disorder that primarily affects the hair follicle. Systemic corticosteroids and methotrexate (MTX) are among the therapeutic options in severe cases. This study aimed to show whether the combination therapy of methylprednisolone (MP) and MTX was superior to MP alone in the management of extensive AA. A total of 26 patients with extensive AA, 14 treated with MP alone and 12 treated with the combination of MP and MTX, were retrospectively evaluated in terms of gender, age, severity of disease, clinical characteristics, disease duration, dose and duration of medications, therapy response, and side effects. Of the 26 patients with extensive AA, 14 were male and 12 were female, and the average age was 17.02 +/- 10.70 years. All patients had more than 50% hair loss, 23 had extensive multifocal AA, and three had alopecia totalis. A total of 14 patients were treated with MP alone (starting dose: 0.3-0.5 mg/kg, maximum 32 mg/day), and 12 were treated with MP + MTX (starting dose: 5-15 mg/week, maximum 20 mg/week). A total of 12 of the 14 patients (85.7%) who were treated with MP alone showed a complete response, with the response rate of the patients who showed more than 50% response being 92.85%. Seven of the 12 patients (58.3%) who were treated with MP + MTX achieved complete healing, and all patients on this regimen had more than 50% treatment response. Our results showed that the combination therapy of MP and MTX was not superior to MP alone in the management of extensive alopecia areata

Internalized Stigma in Hidradenitis Suppurativa: A Multicenter Cross-Sectional Study

Background: Hidradenitis suppurativa (HS) causes significant physical, social, and psychological burdens. Internalized stigma, acceptance of negative attitudes and stereotypes of society regarding a person's illness, has not been studied previously in HS. Objectives: The objective of this study was to investigate the internalized stigma state of HS patients and identify the factors affecting it. Methods: This multicenter, prospective, cross-sectional study included 731 patients. Internalized Stigma Scale (ISS), Hurley staging, Physician Global Assessment, Dermatology Life Quality Index (DLQI), Skindex-16, Beck Depression Inventory-II (BDI-II), and Visual Analog Scale (VAS)-pain score were used in the study. Results: The mean ISS value (57.50 ± 16.90) was comparable to the mean ISS values of studies in visible dermatological and various psychiatric diseases. A significant correlation was found between the mean values of ISS and all disease activity scores, quality of life measures, BDI-II, and VAS-pain scores. Obesity, family history, low education and income level, vulva/scrotum involvement and being actively treated are significant and independent predictive factors for high internalized stigma in multivariate analysis. Conclusions: HS patients internalize society's negative judgements, which may create a profound negative effect on access to health care. Therefore, in addition to suppressing disease activity, addressing internalized stigma is fundamental for improving health care quality

Internalized stigma in Hidradenitis Suppurativa; A multicenter cross-sectional study

Background: Hidradenitis suppurativa (HS) causes significant physical, social, and psychological burdens. Internalized stigma, acceptance of negative attitudes and stereotypes of society regarding a person's illness, has not been studied previously in HS. Objectives: The objective of this study was to investigate the internalized stigma state of HS patients and identify the factors affecting it. Methods: This multicenter, prospective, cross-sectional study included 731 patients. Internalized Stigma Scale (ISS), Hurley staging, Physician Global Assessment, Dermatology Life Quality Index (DLQI), Skindex-16, Beck Depression Inventory-II (BDI-II), and Visual Analog Scale (VAS)-pain score were used in the study. Results: The mean ISS value (57.50 ± 16.90) was comparable to the mean ISS values of studies in visible dermatological and various psychiatric diseases. A significant correlation was found between the mean values of ISS and all disease activity scores, quality of life measures, BDI-II, and VAS-pain scores. Obesity, family history, low education and income level, vulva/scrotum involvement and being actively treated are significant and independent predictive factors for high internalized stigma in multivariate analysis. Conclusions: HS patients internalize society's negative judgements, which may create a profound negative effect on access to health care. Therefore, in addition to suppressing disease activity, addressing internalized stigma is fundamental for improving health care quality

Clinical and demographic features of hidradenitis suppurativa: A multicentre study of 1221 patients with an analysis of risk factors associated with disease severity

Background Hidradenitis suppurativa (HS) is a chronic, relapsing and debilitating inflammatory disease associated with profound morbidity. Aim In this multicentre study, we investigated the demographic and clinical features of HS, and determined risk factors of disease severity. Methods In total, 1221 patients diagnosed with HS from 29 centres were enrolled, and the medical records of each patient were reviewed. Results The mean age of disease onset was 26.2 +/- 10.4 years, and almost 70% (n = 849) of patients were current or former smokers. Mean disease duration was 8.9 +/- 8.4 years with a delay in diagnosis of 5.8 +/- 3.91 years. Just over a fifth (21%; n = 256) of patients had a family history of HS. The axillary, genital and neck regions were more frequently affected in men than in women, and the inframammary region was more frequently affected in women than in men (P < 0.05 for all). Acne (40.8%), pilonidal sinus (23.6%) and diabetes mellitus (12.6%) were the most prevalent associated diseases. Of the various therapies used, antibiotics (76.4%) were most common followed by retinoids (41.7%), surgical interventions (32.0%) and biologic agents (15.4%). Logistic regression analysis revealed that the most important determinants of disease severity were male sex (OR = 2.21) and involvement of the genitals (OR = 3.39) and inguinal region (OR = 2.25). More severe disease was associated with comorbidity, longer disease duration, longer diagnosis delay and a higher number of smoking pack-years. Conclusions Our nationwide cohort study found demographic and clinical variation in HS, which may help broaden the understanding of HS and factors associated with disease severity

A multicentre prospective analysis of the incidence of pemphigoid diseases in Turkey

Background The differentiation between the pemphigoid diseases is essential for treatment and prognosis. In Turkey, data on the incidence of these diseases are insufficient. Our aim in this study is to determine the incidence, demographics and clinical characteristics associated with diseases of the pemphigoid group. Methods We prospectively analysed 295 patients with pemphigoid who visited dermatology clinics of tertiary referral hospitals in 12 different regions of Turkey within a year. The diagnosis was based on clinical, histopathological, direct immunofluorescence (DIF) and serological (multivariant enzyme-linked immunosorbent assay [ELISA], indirect immunofluorescence and mosaic-based BIOCHIP) examinations. Clinical and demographic findings, aetiological factors and concomitant diseases observed in the patients were recorded. Results A total of 295 (female/male ratio: 1.7/1) patients with pemphigoid were diagnosed in 1-year period. The overall incidence rate of pemphigoid diseases was found to be 3.55 cases per million-years. The ratio of pemphigoid group diseases to pemphigus group diseases was 1.6. The most common pemphigoid type was bullous pemphigoid (BP, 93.2%). The others were epidermolysis bullosa acquisita (3.1%), pemphigoid gestationis (2.4%), linear IgA disease (1%) and mucous membrane pemphigoid (0.3%). The most common (26.8%) possible trigger of the bullous pemphigoid was gliptin derivative drugs. The most common concomitant diseases with pemphigoid were cardiovascular (27.8%) and neurological diseases (23.7%). Conclusions This study showed that the increased frequency of bullous pemphigoid reversed the pemphigoid/pemphigus ratio in Turkey. Further studies are warranted regarding the reasons for this increase