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.

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.

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.

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.

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.

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.

Efficacy of peeling during different periods of the menstrual cycle on acne.

BACKGROUND AND DESIGN: The aim of this study was to investigate the efficacy of 50% glycolic acid peeling performed at different phases of menstruation on acne. MATERIALS AND METHODS: This study included 30 patients with mild-to-moderate acne. Those with regular menstrual cycles and no history or laboratory evidence of hormonal pathology, hirsutism were selected. Thirty patients were divided in three groups. The first group received peeling applications in the first 7 days of menstruation; the second group received the peel between 10 and 14 days; and the third group received the peel during the last 10 days of menstruation. RESULTS: The 30 female patients included in study. All patients' menstrual cycles were regular. All groups were homogenous in terms of initial acne severity scores. Acne severity scores decreased in all groups after 3 months of therapy; statistically significant differences were achieved only in the second group. DISCUSSION: The results of our study suggest that chemical peeling administered during ovulation provides the most significant benefit for acne lesions. Ovulation is the period when estrogen reaches its highest level. Estrogen decreases sebum production through different mechanisms. The beneficial effects of estrogen on acne and healing in combination with those of chemical peeling may cause synergistic therapeutic effects with pronounced results.

Prevalence of obesity in paediatric psoriasis and its impact on disease severity and progression.

BACKGROUND/OBJECTIVES: The current literature suggests there is a possible connection between paediatric psoriasis and obesity. However, there is a paucity of research on the influence of increased adiposity on the severity of paediatric psoriasis and disease progression. We aimed to compare the prevalence of being overweight or obese in paediatric psoriasis patients and controls and assess the potential impact of being overweight/obese on disease severity and progression of disease. METHODS: This multicentre prospective case-control study included 289 psoriasis patients (aged < 18 years) treated and followed up by one of the four university hospitals in Turkey. The control group consisted of 151 consecutive age-matched and sex-matched children who lacked a personal or family history of psoriasis. The participants' characteristics, psoriasis-related parametres (e.g., initial subtype, psoriasis area and severity index, presence of psoriatic arthritis) and body mass index were determined. RESULTS: The difference between the prevalence of being overweight/obese among psoriatics (28%) and the control group (19%) was significant (P = 0.024). Being overweight/obese had no significant impact on disease severity and unresponsiveness to topical treatment. Within a median follow-up time of 12 months, 23% of our patients with localised disease at disease onset progressed to generalised disease. The impact of being overweight/obese on disease progression was found to be non-significant; however, disease duration was found to have a significant impact on disease progression (P = 0.026). CONCLUSIONS: Although it is not associated with disease severity and course, increased bodyweight may be a health problem for psoriatic children.

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.

Relationship of psoriatic arthritis with nail and scalp involvement in Turkish psoriasis patients: Multicentered cross-sectional study.

Psoriasis is a common multisystem inflammatory disease, and arthritis is an essential component of the disorder, requiring early diagnosis and prompt treatment for successful management. In this study, we aimed to investigate the relationship between nail and scalp involvement and other covariates with psoriatic arthritis (PsA). This cross-sectional study, conducted from June 2021 through December 2021, included 763 patients from 11 different centers in Turkey. The severity of involvement was evaluated using psoriasis area severity index (PASI), nail psoriasis severity index (NAPSI), and psoriasis scalp severity index (PSSI) scores. Predictors for PsA were evaluated using univariate and multivariate logistic regression models. PsA (n = 155, 21.5%) was significantly more common in patients having a family history of psoriasis (43.2% vs 30.9%, P = .004), nail involvement (68.4% vs 52.3%, P < .001), and coexistence of nail and scalp involvement (53.7% vs 39.6%, P = .002). Furthermore, patients with PsA had considerably higher PASI (7 vs 5.6, P = .006), NAPSI (5 vs 2, P < .001), and PSSI scores (7 vs 4, P = .002) and longer disease duration (months) (126 vs 108, P = .009). In multivariate analysis, female gender [OR: 3.01, 95% CI (1.861-4.880), P < .001], nail involvement [OR: 2.06, 95% CI (1.293-3.302), P = .002)], and body mass index (BMI) [OR: 1.06, 95% CI (1.017-1.100), P = .005] were identified as independent predictors for PsA. Female gender, nail involvement, and high BMI are significant predictors for PsA and warrant detailed rheumatological assessment. Notably, being female is the strongest predictor of increased risk of PsA in our survey. Scalp involvement appears not to be associated with PsA. Also, the presence of PsA seems related to a more severe skin involvement phenotype.

Tumor necrosis factor-alpha antagonist therapy-induced psoriasis in Turkey: analysis of 514 patients.

OBJECTIVES: New adverse events are being reported with the increased use of anti-tumor necrosis factor (TNF) α therapy. We studied cases of anti-TNFα-induced psoriasis observed in our pool of 514 patients receiving anti-TNFα treatment in Turkey. METHODS: Three rheumatoid arthritis patients and 3 ankylosing spondylitis patients with anti-TNFα-induced psoriasis were included in the study. All patients were examined by a dermatologist, and 3 patients underwent skin biopsy. RESULTS: None of the 6 patients had preexisting psoriasis or a familial history of psoriasis. The earliest and latest occurrences of psoriatic lesions were at the 6th week and 44th month of anti-TNFα therapy, respectively. Psoriasis was severe and refractory in two patients (requiring systemic treatment), while it presented as mild in four patients. Anti-TNFα therapy was totally withdrawn in case 1. In case 2, the treatment was halted for 3 months then switched to another TNFα blocker, and case 3 was switched to another anti-TNFα treatment. The treatment was sustained in the other 3 patients (cases 4, 5, and 6). CONCLUSIONS: TNFα blockers are very effective agents in the treatment of psoriasis, but it is interesting that the same molecules can, paradoxically, induce psoriasis. The occurrence of anti-TNFα-induced psoriasis in six out of 514 patients suggests that the incidence of this adverse reaction is, in fact, as not low as presumed in the literature. In some cases, a severe course of psoriasis may limit the use of these agents.

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.

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.

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.

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.

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.

Cutaneous heterotopic bronchogenic tissue in the scapular area.

Cutaneous bronchogenic anomalies are very rare lesions, and most of them present as cysts and/or sinus tracts in the subcutaneous layer. We report a 15-year-old boy who presented with a crusted and papillomatous plaque over the right scapular region. Histopathological examination of this plaque revealed pseudostratified ciliated columnar epithelium with papillary invaginations that included mucinous material. The lesion had no cyst or sinus formation and was bordered with smooth muscle bundles in stroma.