Evaluation of the Factors Influencing Mortality in Patients with Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A Multicenter Study of 166 Patients.

INTRODUCTION: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening acute mucocutaneous disorders usually triggered by drugs. In this study, we aimed to evaluate the factors affecting mortality in patients with SJS-TEN. METHODS: Our study is a retrospective cohort study, analyzing data collected from a total of 12 tertiary care centers between April 2012 and April 2022. RESULTS: The study included 59 males and 107 females, a total of 166 patients, with an average age of 50.91 ± 21.25 years. Disease classification was TEN in 50% of cases, SJS in 33.1%, and SJS-TEN overlap in 16.9%. The average SCORTEN within the first 24 h was 2.44 ± 1.42. Supportive care was provided to 99.4% of patients. The most commonly used systemic immunomodulatory treatments were systemic steroids (84.3%), IVIG (intravenous immunoglobulin) (49.3%), and cyclosporine (38.6%). Plasmapheresis was administered to five patients. While 66.3% of patients were discharged, 24.1% resulted in exitus. Our comparative analysis of survivors and deceased patients found no effect of systemic steroids, IVIG, and cyclosporine treatments on mortality. Univariate analysis revealed that the SCORTEN scores on days 1 and 3 as well as the rates of detachment at the onset and during follow-up were significantly higher in deceased patients compared to survivors. The rates of fever, positive blood cultures, and systemic antibiotic use were higher in deceased patients compared to survivors. The presence of comorbidities, diabetes, and malignancy were significantly more common in deceased patients. Multivariate regression analysis indicated that over SCORTEN 2, the mortality risk exponentially rose with each SCORTEN increment, culminating in an 84-fold increase in mortality at SCORTEN 5-6 (odds ratio [95% confidence interval]: 13.902-507.537, p < 0.001) compared to SCORTEN 0-1. Additionally, the utilization of plasmapheresis was associated with a 22-fold increase in mortality (odds ratio [95% confidence interval]: 1.96-247.2, p = 0.012). CONCLUSION: Our study found that a high SCORTEN score within the first 24 h and the use of plasmapheresis were related to increased mortality, while systemic steroids, IVIG, and cyclosporine treatments had no impact on mortality. We believe that data gathered from one of the most comprehensive studies which we conducted on SJS-TEN will enrich the literature, although additional research is warranted.

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.

Factors Related to Omalizumab Drug Survival and Treatment Responses in Chronic Urticaria.

INTRODUCTION: This study aimed to evaluate factors affecting drug survival and treatment response in patients with chronic urticaria treated with omalizumab in clinical practice. METHODS: This study included 386 patients with chronic urticaria. Demographic characteristics, clinical features, laboratory parameters, and omalizumab treatment data were analyzed retrospectively. The 7-day urticaria activity score (UAS7) and urticaria control test (UCT) were used to assess disease severity and treatment responses. RESULTS: Well-controlled disease (UAS7 ≤6) was achieved in 59.3% of patients at a median of 2 months. Complete response was significantly higher in patients treated with omalizumab for ≥12 months (p < 0.001). Family history of asthma (p = 0.01) was less frequent, and disease duration (p = 0.041) was shorter in patients with well-controlled disease. Total treatment duration was longer in patients with relapse (p < 0.001) and serum Helicobacter pylori IgA positivity (p = 0.029). DISCUSSION/CONCLUSION: Treatment response is better in patients treated with omalizumab for ≥12 months. However, prolonged treatment does not prevent relapse. Our findings suggest that continuous or intermittent therapy is an appropriate alternative treatment option in patients with severe chronic urticaria; however, continuous therapy can be preferred to maintain the patient's quality of life.

Omalizumab versus cyclosporin-A for the treatment of chronic spontaneous urticaria: can we define better-responding endotypes?

BACKGROUND: Chronic Spontaneous Urticaria (CSU) is characterized by recurrent wheals and/or angioedema for longer than 6-weeks. Guidelines recommend Omalizumab (Oma) as first-line and Cyclosporine-A (Cs-A) as second-line treatment in antihistamine resistant CSU. This step-wise algorithm might be time-consuming and costly. OBJECTIVE: To determine indicators of response to Oma or Cs-A in CSU patients. METHODS: We retrospectively analyzed data from seven centers in Turkey; the inclusion criteria for patients were to receive both Oma and Cs-A treatment (not concurrently) at some point in time during their follow-up. Clinical and laboratory features were compared between groups. RESULTS: Among 110 CSU patients; 47 (42.7%) were Oma-responders, 15 (13.6%) were Cs-A-responders, and 24 (21.8%) were both Oma and Cs-A responders and 24 (21.8%) were non-responders to either drug. High CRP levels were more frequent in Cs-A-responders (72.7% vs. 40.3%; p = 0.055). Oma-responders had higher baseline UCT (Urticaria Control Test) scores (6 vs. 4.5; p = 0.045). Responders to both drugs had less angioedema and higher baseline UCT scores compared to other groups (33.3% vs. 62.8%; p = 0.01 and 8 vs. 5; p = 0.017). Non-responders to both drugs had an increased frequency in the female gender and lower baseline UCT scores compared to other groups (87.5% vs. 61.6%; p = 0.017 and 5 vs. 7; p = 0.06). STUDY LIMITATIONS: Retrospective nature, limited number of patients, no control group, the lack of the basophil activation (BAT) or BHRA (basophil histamine release assay) tests. CONCLUSIONS: Baseline disease activity assessment, which considers the presence of angioedema and disease activity scores, gender, and CRP levels might be helpful to predict treatment outcomes in CSU patients and to choose the right treatment for each patient. Categorizing patients into particular endotypes could provide treatment optimization and increase treatment success.

Birt-Hogg-Dubé Syndrome: Diagnostic Journey of Three Cases from Skin to Gene.

Birt-Hogg-Dube syndrome (BHDS) is a rare disorder characterized by the triad of cutaneous lesions, renal tumors, lung cysts and inactivation of the gene folliculin (FLCN). Here, we present three female patients diagnosed with BHDS. First case a 55-year-old female had flesh moles histopathology compatible with angiofibroma, multiple cysts in the lung and kidneys, FLCN gene mutations ('c.1285dupC [p.His429Profs*]' 11th exon and 'c.653G>A [p.Arg258His]' 7th exon). The second case a 76-year-old female had trichodiscoma on her skin, multiple cysts in the lung, spontaneous pneumothorax, FLCN gene mutation 'c.1285dupC (p.His429Profs*27) 11th exon' and, her son had renal carcinoma history under 50 years of age. Our third case, also the daughter of case 2, had dermal papules histopathology compatible with trichodiscoma, spontaneous pneumothorax, FLCN gene mutation 'c.1285dupC (p.His429Profs*27) 11th exon' and, parotid oncocytoma. Through our cases, we document the first case of two mutations ('c.1285dupC [p.His429Profs*]' 11th exon and 'c.653G>A [p.Arg258His]' 7th exon) in the same FLCN gene and the 11th known case of parotid oncocytoma associated with BHDS in the light of the literature.

Predictors of drug survival of biologic therapies in psoriasis patients.

INTRODUCTION: We aimed to investigate the clinical, immunological, and genetic factors affecting the response to anti-TNFα (tumor necrosis factor-α) and interleukin-12/23 therapies and drug survivals. METHODS: A total of 180 patients were divided into two groups: 89 patients who used at least two biologic agents, with the initial biologic agent used less than 12 months (group A), and 91 biologic-naive patients who have been receiving a single biologic agent for more than 12 months (group B). ELISA (enzyme-linked immunosorbent assay) was used to analyze anti-drug antibodies (ADAs) in blood samples. Clinical data of the patients were retrospectively analyzed. HLA-SSO (sequence-specific oligonucleotide) Typing Kits were used for HLA-C typing. IBM SPSS v.21 was used for statistical analysis.Results: Infliximab had the longest drug survival as the first biologic agent in group A (p = .015). Etanercept had the lowest ADA count compared to the other anti-TNF agents (p = .001). HLA-Cw6 negativity, late-onset psoriasis, smoking and alcohol use were determined to be risk factors for treatment failure in group A. HLA-Cw6 was found to be associated with type I psoriasis (p = .000). CONCLUSIONS: Although our study is retrospective of a relatively low number of patients, this is a preliminary study focusing on two different patient populations based on therapy response.

Assessment of disease activity and quality of life in patients with recurrent bradykinin-mediated versus mast cell-mediated angioedema.

OBJECTIVE: Recurrent Angioedema (RAE) is characterized by sudden swelling of mucosal surfaces or deep dermis and is either mast cell-(MMAE) or bradykinin-mediated (BMAE). How patients with BMAE and MMAE differ in terms of disease activity and impact remains largely unknown. Here, we determined validity, reliability, and sensitivity to change of Turkish versions of angioedema activity score (AAS) and quality of life questionnaire (AE-QoL) and used both instruments to investigate and compare patients with BMAE and MMAE. METHODS: Turkish versions of AAS28 and AE-QoL were applied to 94 patients with RAE (18-72 years). Patients' global self-assessment of QoL (PGA-QoL), disease activity (PGA-DA-VRS, PatGA-DA-VAS), and 12-Item-Short Form Survey were used at week 4 (visit 2), and week 8 (visit 3). Demographic characteristics, clinical features, and AAS28 and AE-QoL values were compared between 31 patients with BMAE and 63 patients with MMAE. RESULTS: Turkish AAS28 and AE-QoL showed excellent internal consistency, high reproducibility and known-groups validity. Compared to patients with MMAE, BMAE patients were younger (34.6 ± 10.7 vs. 40.7 ± 13.3 years), had longer disease duration (236 ± 178 vs. 51 ± 78 months), high prevalence of family history (63% vs 14%), longer duration of attacks (65 ± 20 vs. 40 ± 25 h), and they were more commonly affected by upper airway angioedema (70% vs 23%). Disease activity (AAS28) was lower (29.3 ± 24.6 vs 55.2 ± 52.9), but AE-QoL was higher (44.2 ± 16.1 vs 34.5 ± 22.5) in BMAE patients as compared to MMAE patients. CONCLUSIONS: Patients with BMAE and MMAE have distinct disease characteristics. Recurrent bradykinin-mediated angioedema impacts quality of life more than mast cell-mediated angioedema. The discriminating characteristics of patients with BMAE and MMAE may help to improve the diagnosis and management of patients with RAE.

Patch Test Results to European Baseline Series in Turkey: A Prospective and Multicenter Study.

BACKGROUND: Patch testing with the European Baseline Series (EBS) is an essential diagnostic tool for the assessment of allergic contact dermatitis. OBJECTIVE: The aims of the study were to describe the most common contact allergens identified with patch testing in Turkey and to establish the distribution of contact allergens among different regions in Turkey. PATIENTS AND METHODS: Twelve centers consisting of tertiary health care institutions were included in the study. A total of 1169 patients were patch tested with the EBS from 2015 to 2017. RESULTS: A total of 596 patients (51.0%) had 1 or more positive patch test reactions to the EBS. A total of 30.2% (n = 353) of those tested had clinical relevance. Contact sensitivity was more common in young patients. The most common 10 allergens were nickel sulfate (20.4%), followed by textile dye mix (8.6%), cobalt chloride (8.3%), potassium dichromate (8.0%), p-phenylenediamine base (4.5%), balsam of Peru (4.3%), methylchloroisothiazolinone/methylisothiazolinone (3.8%), thiuram mix (3.6%), fragrance mix I (3.0%), and methylisothiazolinone (2.9%). CONCLUSIONS: This study provides a comprehensive profile of patch test results with the EBS in Turkey. An ongoing decrease in contact allergy to methylchloroisothiazolinone/methylisothiazolinone and methylisothiazolinone was observed. Textile dye mix and p-phenylenediamine are beginning to pose a new risk in Turkey.

Psoriasis and 5HT-R2C Gene Polymorphism: Association between Clinical, Demographic and Therapeutic Parameters in the Turkish Population.

We aimed to investigate the relationship between single nucleotide polymorphism (SNP) in the promoter region of the 5-HT-R2C gene and stress-related disease psoriasis in the Turkish population. The putative association between the 5-HTR2C variant (rs6318 Cys23Ser allele) and patients with psoriasis was investigated. 100 patients with psoriasis and 100 age-sex matched, unrelated healthy subjects representing the control group were included in the study. The PCR-RFLP method was used for genotyping the 5-HTR2C variation. There was no statistically difference in terms of genotype distributions and allele frequencies between the control subjects and patients with psoriasis (P=0.360 and P=0.439, respectively). The comparison between the presence and absence of the 5-HTR2C gene rs6318 G allele within the determined clinical subsets resulted in a significant difference with regard to treatment methodology only when conventional therapy and one or more medical therapy was compared (P=0.021). This study is the first clinical study to investigate the association between 5-HTR2C polymorphism and psoriasis. The role of the 5-HTR2C gene should be examined with more parameters in a larger case series.

Clinical and Demographical Characteristics of Familial Behçet's Disease (Southeast Marmara Region).

BACKGROUND: Familial aggregation in Behçet's disease (BD) has been reported in Turkish and Japanese populations. While the frequency of familial cases has been reported to be 2-5% worldwide, this rate reaches up to 15% in the Middle East. OBJECTIVE: This study aimed to determine the incidence of familial BD cases followed in the BD polyclinic and to compare their clinical and demographic characteristics to those observed in sporadic cases. METHODS: Data related to BD patients who were followed between 1995 and 2014 were collected from computerized archive records and were assessed for detailed family histories. Only first-degree relatives (brother, sister, mother, father, children) were considered to be cases of familial BD. Clinical and demographic -features were retrieved. Our BD polyclinic is located in the Southeast Marmara Region in Turkey. RESULTS: BD was detected in 36 first-degree relatives of 33 patients out of 840 patients with BD. A total of 45 patients were diagnosed as familial BD;23 were female, and 22 were male. In our patients, the incidence of familial BD was determined to be 3.9%. The rates for HLA-B5 positivity, ocular involvement, genital ulcers, and erythema nodosum were determined to be 86.6% (26/30), 26.6%, 82.2%, and 60%, respectively. None of the patients had neurological involvement, but 2 had vascular involvement. CONCLUSION: This study may contribute to the epidemiological data of BD from Turkey.

A Report of 13 Cases of Merkel Cell Carcinoma: Single-Center Experience and Review of the Literature.

BACKGROUND: There is limited data knowledge of Merkel cell carcinoma (MCC) in Turkey aside from a few case reports. OBJECTIVE: The aim of this study was to describe the clinical characteristics, demographic features, therapeutic parameters, and outcome of primary cutaneous MCC cases from Turkey. METHODS: Digital medical records of the 13 MCC patients who were followed-up at a tertiary referral center were retrospectively analyzed. Clinic, demographic, tumor characteristics, and survival of the patients were retrieved. RESULTS: Most of our patients were elderly. Female predominance was noticed. The most common primary site of the tumors was the lower extremities. The overall survival was 42 months, 68% at first year, 68% at third years, and 29% at fifth years. CONCLUSION: This is the first largest report from Turkish population with female predominance, and lower extremity tendency.

The effect of personal, familial, and environmental characteristics on acne vulgaris: a prospective, multicenter, case controlled study.

BACKGROUND: There are only a few studies about epidemiological features of acne vulgaris in the literature. The aim of this study was to analyze demographic, clinical, familial and environmental characteristics of acne, the role of diet and aggravating factors and association of these factors with acne severity. METHODS: Patients with a diagnosis of mild-moderate to severe acne were consecutively interviewed at the participating centers during the study period. RESULTS: A total of 3826 patients and 759 control patients were involved in this study. Mild acne was the most common type of acne, and most of the lesions were localized on face followed by the trunk. The severity of acne was worse in patients who had a positive family history of acne. The most common triggering factor was psychological stress. We found a positive correlation with chocolate, bread, green tea, milk, white sugar, ripe banana, ice cream, apple, orange, and red meat consumption. As we compare the acne severity according to geographical features we detected mild- moderate acne was more common in Mediterranean region and severe acne was more common in East Anatolian region. Family history positivity was more common in Aegean region and least common in Central Anatolian region. There was statistically significant relationship as we compare acne severity and dietary factors such as chocolate, dairy products such as milk, sunflower seed consumption within the geographical regions. CONCLUSIONS: This study presents the demographic and clinical characteristics of acne patients in Asian and the European parts of Turkey. We believe that this study will provide a useful overview of acne in Turkey.

Evaluation of the efficacy of microneedle fractional radiofrequency in Turkish patients with atrophic facial acne scars.

BACKGROUND: Scarring is an undesirable and severe complication of acne resulting in loss of self-esteem in young people. Although microneedle fractional radiofrequency (MFR) system has emerged as a good option to treat acne scars in recent years, it was examined in a few studies which were commonly from Asian countries. AIMS: We sought to evaluate the efficacy of MFR in Turkish patients with facial acne scars. METHODS: Nine patients with atrophic facial acne scars treated with MFR device were included in the study. The number of treatment sessions was varied from one to five (median three) with 4-week intervals. Demographic and basal clinical features were recorded. Efficacy of the device was evaluated by the physicians' global assessment and patients' self-assessment scales 4 weeks after the last treatment session. RESULTS: Of nine patients, two were male and seven were female (mean age, 31.33 years). Two patients had mild, four had moderate, and three had severe facial acne scars. Mean acne scar age was 13.22 ± 8.79 years. According to the predominant scar subtype, three patients had V-shaped, three had U-shaped, and three had M-shaped atrophic acne scars. A clinical improvement of >25% has been reported in seven patients (77.7%) and eight patients (88.9%) by the physicians and patients, respectively. U-shaped atrophic acne scars responded better to the treatment than the other types, as statistically nonsignificant. There were no severe side effects. CONCLUSIONS: Microneedle fractional radiofrequency system showed a quite good efficacy and safety in the treatment of atrophic facial acne scars (Department of Dermato-Cosmetology, Uludag University Medical School).

Evaluation of long-term efficacy, safety, and effect on life quality of pulsed dye laser in rosacea patients.

Background: Rosacea is a chronic disease affecting the patients' life quality negatively. Although various laser systems are used in the rosacea treatment, studies reporting efficacy and long-term continuity of benefit of laser therapies are scarce. Objectives: We aimed to evaluate the efficacy, safety, continuity of benefit, and effect on life quality of pulsed dye laser (PDL) in the rosacea patients. Methods: Fourteen rosacea patients treated with PDL were enrolled in the study. The number of treatment sessions were varied from one to four. The efficacy was evaluated by the physicians' clinical assessment (PCA), patients' self-assessment (PSA), and erythema and telengiectasia grading scores. Additionally, the patients were asked about the continuity of the benefit and improvement in their life qualities after the treatment. Results: Both the erythema and telangiectasia scores were significantly improved after the treatment (p < 0.001). According to PCA, nine patients had a clinical improvement of >50%. According to PSA, 11 patients had good/excellent improvement. Moderate/significant benefit of treatment continued in 12 patients at the follow-up period (mean 21.64 ± 14.25 months). The life quality scores were significantly improved. No serious side effects were observed. Conclusion: PDL has high and long-term efficacy in the treatment of rosacea with a good safety profile.

The Effect of Single-Dose Intraperitoneal Bevacizumab on Peritoneal Adhesion Formation.

BACKGROUND: Intra-abdominal adhesions and their complications following abdominal surgery are serious problems, with an incidence of 67-93%. Prevention of peritoneal adhesion formation may eliminate the need for surgical intervention, decreasing complications, morbidity, and cost. Bevacizumab is a recombinant monoclonal antibody which specifically binds vascular endothelial growth factor, an important cytokine in adhesion formation, and neutralizes its biological activity. We developed an experimental model in rats to determine the effect of bevacizumab in preventing adhesion formation and analyzed its effect both micro- and macroscopically. METHODS: We used 32. Wistar rats randomly divided into two groups: Group A (control) and Group B (bevacizumab), with 16 rats each. A modified cecum abrasion model was developed; 0.9% NaCl solution was administered intraperitoneally to Group A and bevacizumab to Group B. On day 15, adhesion formation was evaluated both macro- and microscopically. RESULTS: Both micro- and macroscopic adhesion grades in Group B were significantly lower than those of control Group A; macroscopic grades were 2.69 ± 0.95 and 0.69 ± 0.8, and microscopic grades were 2.25 ± 1.06 and 0.5 ± 0.52 for Groups A and B, respectively. CONCLUSIONS: Bevacizumab was effective in preventing intraperitoneal adhesion formation in our study; however, its inhibitory effects on embryogenesis and the hematopoietic, endocrine, and immune systems may limit its clinical use.

Cyclosporine-A for severe childhood atopic dermatitis: clinical experience on efficacy and safety profile

Background/aim: Management of atopic dermatitis (AD) in children is still challenging. The aim of this study was to evaluate the efficacy and safety profile of cyclosporine-A (CsA) treatment in children with severe and recalcitrant AD. Materials and methods: Medical records of 43 children followed between January 2010 and December 2015 and treated with systemic CsA were evaluated retrospectively. Treatment efficacy was assessed according to the physician's global assessment (PGA) score. According to the treatment response, patients were grouped as nonresponder, moderate responder, or good responder. Effects of the variables on treatment response were evaluated by analysis of variance (ANOVA). The safety profile of CsA was assessed by clinical and laboratory findings at each visit. Results: The median initial dose of CsA was 3 mg/kg daily, ranging between 2.5 and 5 mg/kg daily. The mean duration of CsA therapy was 4.9 ± 4.24 months. Seventeen patients (39.5%) achieved good response in a treatment period of 3 to 14 months. After discontinuation of CsA, of the 17 patients, relapse was observed in 4 (23.5%). Moderate response was observed in 12 (27.9%) patients; however, 14 (32.6) patients did not respond to the treatment. Five patients reported mild side effects. Conclusion: Low-dose CsA seems to be an effective and safe treatment option for severe and recalcitrant AD in children.

Retrospective Analysis of 91 Kaposi's Sarcoma Cases: A Single-Center Experience and Review of the Literature.

BACKGROUND: Kaposi's sarcoma (KS) is a multifocal angioproliferative tumor involving primarily the skin. OBJECTIVE: The aim of this study was to describe the clinical, demographic, histopathological characteristics, treatment modalities, and outcome of 91 KS patients, and compare them with other contemporary research. METHODS: Medical records of 91 KS patients followed between January 2005 and September 2017 were evaluated retrospectively. RESULTS: Most of our patients were male (male-to-female ratio was 4.05). The median age at diagnosis was 69 years (range, 6-93 years). The duration of the lesions varied between 3 and 25 years. The lower extremities were the most commonly involved area (51.6%). Of the 91 patients, classic type KS was seen in 75 patients. Radiotherapy was used successfully in approximately half of our patients. Recurrence was observed in approximately one third of the patients. All KS patients in this study except 1 were classic KS. CONCLUSION: The clinical and demographic characteristics of our patients were compatible with the previous literature suggesting that KS is a tumor that tends to be limited to the skin. Close follow-up of patients is important to monitor for recurrence. This is the largest report from Turkey to date.

The efficacy and safety of omalizumab in refractory chronic spontaneous urticaria: real-life experience in Turkey.

INTRODUCTION: This study used real-world data to evaluate the effectiveness and reliability of omalizumab in treating recalcitrant chronic spontaneous urticaria in Turkish patients. METHODS: Study data were collected retrospectively from eight tertiary-care hospitals in Turkey. This study included 132 patients with chronic spontaneous urticaria that were resistant to H1 antihistamine treatment in a dose up to four times the licensed dose and were treated with 300 mg/month of omalizumab for 6 months. RESULTS: The mean weekly urticarial activity score (UAS7) after omalizumab treatment improved significantly compared to the pre-treatment score (p < 0.001). Treatment response was detected primarily in the 1st and 2nd months after treatment. No significant association was observed between omalizumab's treatment effectiveness and disease-related parameters or laboratory data. The mean dermatology life quality index was 23.12 ± 6.15 before treatment and decreased to 3.55 ± 3.60 6 months after treatment (p < 0.001). No side effects were reported in 89.4% (118) of the patients. CONCLUSIONS: This study showed that UAS7 decreased significantly and quality of life improved in omalizumab-treated patients. Moreover, treatment effectiveness was mainly observed in the first 2 months after treatment. However, no association was observed between omalizumab treatment effectiveness and disease-related parameters or laboratory data.

Treatment of lichen planopilaris: methotrexate or cyclosporine a therapy?

BACKGROUND: Because of irreversible outcome of the lichen planopilaris (LPP), systemic therapy should be used in early inflammatory stages of the disease, without allowing the irreversible scar formation and permanent hair loss. OBJECTIVE: We assessed the efficacy and safety of methotrexate (MTX) and cyclosporine A (CsA) in the management of recalcitrant, extensive LPP and compared their efficacy and safety profile. METHODS: We retrospectively analysed the 16 LPP cases treated with either CsA or MTX therapy. Clinical improvement was defined as the absence of reported symptoms, lack of progression and reduction in erythema and follicular hyperkeratosis found in SIAscopic images. RESULTS: A total of 16 patients received either CsA (six cases) or MTX (10 cases) therapy. The dosage of CsA was between 3 and 5 mg/kg/day. The initial dosage of MTX was 10-15 mg/wk and tapered gradually. The clinical improvement was demonstrated significantly at SIAscopic images taken at the third months of therapy. CONCLUSIONS: Our observations suggest that both MTX and CsA therapies provide similar clinical efficacy at the end of first month of therapy with dosages used in psoriasis therapy. MTX was found to be better tolerated in this study.

Epidemiology of Pemphigus in Turkey: One-year Prospective Study of 220 Cases.

Pemphigus is a group of rare and life-threatening autoimmune blistering diseases of the skin and mucous membranes. Although they occur worldwide, their incidence shows wide geographical variation, and prospective data on the epidemiology of pemphigus are very limited. Objective of this work is to evaluate the incidence and epidemiological and clinical features of patients with pemphigus in Turkey. All patients newly diagnosed with pemphigus between June 2013 and June 2014 were prospectively enrolled in 33 dermatology departments in 20 different provinces from all seven regions of Turkey. Disease parameters including demography and clinical findings were recorded. A total of 220 patients were diagnosed with pemphigus during the 1-year period, with an annual incidence of 4.7 per million people in Turkey. Patients were predominantly women, with a male to female ratio of 1:1.41. The mean age at onset was 48.9 years. Pemphigus vulgaris (PV) was the commonest clinical subtype (n=192; 87.3%), followed by pemphigus foliaceus (n=21; 9.6%). The most common clinical subtype of PV was the mucocutaneous type (n=83; 43.2%). The mean Pemphigus Disease Area Index was 28.14±22.21 (mean ± Standard Deviation). The incidence rate of pemphigus in Turkey is similar to the countries of South-East Europe, higher than those reported for the Central and Northern European countries and lower than the countries around the Mediterranean Sea and Iran. Pemphigus is more frequent in middle-aged people and is more common in women. The most frequent subtype was PV, with a 9-fold higher incidence than pemphigus foliaceus.