Evaluation of the Quality of Life and the Demographic and Clinical Characteristics of Patients With Pemphigus With Oral Mucosal Involvement: A Multicenter Observational Study.

INTRODUCTION: Pemphigus vulgaris (PV) is an autoimmune disease primarily affecting the oral mucosa. OBJECTIVES: This study aimed to determine the demographic, clinical and treatment characteristics of PV patients with oral mucosal involvement and to assess the impact on their quality of life. METHODS: We conducted a prospective observational study among 106 patients diagnosed with PV and presenting oral mucosal involvement. Demographic data, clinical and treatment characteristics, and quality of life questionnaires were recorded. RESULTS: The study included 106 patients, 55 (51.89%) were male and there was a predominance of the mucocutaneous subtype in 83 individuals (78.38%). Oral mucosa was the initial site of manifestation in 44 patients (41.51%). Bilateral buccal mucosa was the most frequently affected site. The predominant symptom reported was a burning sensation, noted in 91 patients (85.85%). Oral mucosal examination revealed erosions in 85.85% of the patients. Systemic steroids were the most commonly administered treatment, and rituximab was used in 18 patients (16.98%). A positive and significant correlation was found between pemphigus severity and Oral Health Impact Profile-14, Dermatology Life Quality Index and Dermatological Quality of Life Scale scores (P < 0.05). The presence of superficial ulcers, flaccid bullae, lesion diameter ≥1 cm, and >10 lesions were factors that markedly diminished quality of life. Complete response to treatment was noted in all patients administered rituximab. CONCLUSIONS: The most common area of involvement was bilateral buccal mucosa, and the severity of PV closely correlated with a decline in quality of life measures. These results highlight the need for careful clinical oversight of PV, taking into account its effects on patients quality of life.

Analysis of factors influencing target PASI responses and side effects of methotrexate monotherapy in plaque psoriasis: a multicenter study of 1521 patients.

Methotrexate (MTX) is commonly used as first-line systemic treatment agent in psoriasis. We aimed to evaluate the clinical characteristics and treatment responses of patients with psoriasis undergoing MTX monotherapy. Data from adult patients with plaque psoriasis who received MTX monotherapy for at least 3 months between April 2012 and April 2022 were retrospectively evaluated in 19 tertiary care centers. Our study included 722 female and 799 male patients, a total of 1521 participants. The average age of the patients was 44.3 ± 15.5 years. Mode of treatment was oral in 20.4% of patients while in 79.4% it was subcutaneous. The median treatment duration was 8 months (IQR = 5-15). The median weekly dose was 15 mg (IQR = 11-15). 1448 (95.2%) patients were taking folic acid supplementation. At week 12, 16.3% of the patients achieved PASI (Psoriasis Area and Severity Index) 90 response while at week 24, 37.3% achieved it. Logistic regression analysis for week 12 identified the following independent factors affecting PASI 90 achievement positively: median weekly MTX dose ≤ 15 mg (P = 0.011), subcutaneous administration (P = 0.005), no prior systemic treatment (< 0.001) and folic acid use (0.021). In logistic regression analysis for week 24; median weekly MTX dose ≤ 15 mg (P = 0.001), baseline PASI ≥ 10 (P < 0.001), no prior systemic treatment (P < 0.004), folic acid use (P = 0.001) and absence of comorbidities (P = 0.009) were determined as independent factors affecting the achievement of PASI 90. Adverse effects were observed in 38.8% of the patients, with nausea/vomiting (23.9%) and transaminase elevation (13%) being the most common. The most common reasons for interruptions (15.3%) and discontinuations (27.1%) of the treatment were patient related individual factors. The use of MTX as the first systemic treatment agent, at doses ≤ 15 mg/week and concurrent folic acid application are positive predictive factors for achieving the target PASI response both at weeks 12 and 24. In our study, which is one of the most comprehensive studies on MTX treatment in psoriasis, we observed that MTX is an effective and safe treatment option.

Systemic disease in leukocytoclastic vasculitis: a focus on direct immunofluorescence findings.

BACKGROUND: Direct immunofluorescence (DIF) panels are usually ordered for clinically suspected cutaneous vasculitis, but their positivity rate is variable, and their prognostic significance is not clear to date. OBJECTIVE: The study aims to investigate the systemic involvement rate in leukocytoclastic vasculitis (LCV) patients and the potential clinical and laboratory associations with systemic involvement, including DIF findings. METHODS: A retrospective study of patients with histopathologically proven cutaneous LCV examined in the dermatology department between 2013 and 2017 was performed. RESULTS: Of the 81 patients (mean age, 50.6 years), 42 (52%) were male. The mean time between the appearance of skin lesions and biopsy was 23.1 days, ranging from 2 to 180 days. DIF showed overall positivity of 90.1%, and C3 was the most frequent immunoreactant (82.7%). Any kind of extracutaneous involvement was present in 47 (58%) of patients, with renal involvement being the most frequent (53.1%), followed by articular (18.5%) and gastrointestinal (11.1%) involvement. The presence of renal disease was associated with the detection of IgG in the lesional skin (p = 0.017), and with the absence of IgM in the lesional skin (p = 0.032). There was a significant association between C3 deposition and joint involvement (p = 0.05). STUDY LIMITATIONS: This is a single-center study with a retrospective design. CONCLUSION: DIF seems to be a useful ancillary diagnostic tool in the evaluation of cutaneous vasculitis, but the relationship between DIF findings and systemic involvement needs to be further elucidated due to contradictory data in the current literature.

Evaluation of serum vascular endothelial growth factor level and findings of nailfold capillaroscopy by dermatoscope in the differential diagnosis of palmoplantar psoriasis and palmoplantar eczema.

OBJECT: This study aimed to evaluate VEGF level, capillaroscopic findings, and these features for differential diagnosis of palmoplantar psoriasis and eczema patients. METHODS: This comparative cross-sectional study included 40 patients clinical and histopathologically diagnosed with eczema or psoriasis (20 psoriasis and 20 eczema) and 30 patients of similar age and gender without inflammatory disease in the control group. Serum samples of patients were collected and analyzed for VEGF by enzyme-linked immunosorbent assay (ELISA) and at the same time, we applied capillaroscopy with computerized dermatoscopy qualitatively and semiquantitatively. RESULTS: In the qualitative evaluation, a decrease in capillary density and a significant increase of the avascular area was in palmoplantar plaque psoriasis (PPP) and palmoplantar eczema (PPE) patients compared to the control group. In the semiquantitative evaluation, the mean score decreased capillary density was significantly higher in PPE than in the PPP and control groups (p < 0.001). Minor morphological change (tortuous, crossed and enlarged capillary) scores were significantly higher in patients with PPE compared to PPP and control groups (p = 0.011). Major morphological change (mega, meandering, branching, bushy, bizarre, and disorganized polymorphic capillary) score was significantly higher in patients with PPP than PPE and the control group (p < 0.001). Major morphological change and m-PPPASI scores were correlated in PPP patients (p < 0.05). Disorganized capillaries in PPP patients were significantly higher than in the PPE and control groups (p < 0.001). There was no significant correlation between serum VEGF value and clinical severity and capillaroscopy findings of the patients. CONCLUSION: Significant morphological changes were detected between PPP, PPE, and control groups in the qualitative and semi-quantitative evaluation of capillaroscopic findings. In the differential diagnosis of PPP and PPE, major morphological change, especially the presence of disorganized polymorphic capillaries, was considered an important finding in the capillaroscopic evaluation. These capillaroscopic findings may be helpful to discriminate eczema and psoriasis. However, there was no significant relationship between serum VEGF level and the patients' capillaroscopic findings and clinical severity.

Evaluation of clinicopathological and treatment characteristics of 80 patients with acquired perforating dermatosis.

Acquired perforating dermatosis (APD) is a group of a rare dermatological disorder characterized by elimination of dermal connective tissue through epidermis. We aimed to evaluate the characteristics of patients diagnosed with APD and to determine the differences in comorbidities according to subtypes of APD. A retrospective, observational, cross-sectional study was designed. Patients diagnosed with APD between January 2008 and January 2019 were reviewed. Eighty patients were included in the study. 61.2% (n = 49) of the patients were female and 38.8% (n = 31) were male with a mean age of 58.4 ± 12.5 years. 82.5% (n = 66) of the patients were diagnosed with reactive perforating collagenosis (RPC) and 17.5% (n = 14) of perforating folliculitis (PF). The most common concomitant disease was diabetes mellitus (82.5%). 5.0% of the patients had malignancy. The comorbidity rate in RPC group was higher than PF (P < .05). Topical steroid was the most frequently (90.0%) used treatment. Complete response was obtained 55.0% of patients. Exitus was observed in 23.8% (n = 19) of patients in a mean 17.6 ± 25.7 months follow-up period. APD may be associated with many diseases. Comorbidities are more frequent in RPC group. This situation warns us to evaluate patients with RPC in more detail for underlying diseases. High mortality rate related to the underlying systemic diseases suggests being careful in terms of mortality in patients diagnosed with APD.

Effect of omalizumab use on glucose homeostasis in non-diabetic patients with chronic urticaria.

PURPOSE: In some diabetic patients receiving omalizumab therapy, blood glucose regulation is impaired. However, the effect of omalizumab on glucose homeostasis in non-diabetic patients remains unknown. METHODS: The patients were given subcutaneous omalizumab at a dose of 300 mg every four weeks for the treatment of chronic urticaria in this study. Fasting blood glucose, fasting plasma insulin, total cholesterol, triglyceride, and high-density (HDL) and low-density lipoprotein (LDL) were studied before and at the 12th week of treatment. The homeostatic model assessment for insulin resistance (HOMA-IR) was used to determine insulin resistance. RESULTS: Forty of the 45 patients included in the study completed 12 weeks of treatment. While fasting blood glucoses (p = 0.011) and HOMA-IR values (p = 0.027) were significantly increased in the 12th week, the increase in fasting insulin level was not significant (p = 0.07). After treatment, 10 patients developed impaired fasting glucose and 13 developed insulin resistance. CONCLUSION: The increase in blood glucose levels may be associated with the paradoxically increase of histamine levels in the blood by omalizumab. If this increase cannot be balanced with insulin, patients may develop impaired glucose tolerance and insulin resistance. Therefore, we suggest that patients using omalizumab should be followed up for glucose homeostasis and insulin resistance.

Are clinicians successful in diagnosing cutaneous adnexal tumors? a retrospective, clinicopathological study

Background/aim: Cutaneous adnexal tumors (CAT) are rare tumors originating from the adnexal epithelial parts of the skin. Due to its clinical and histopathological characteristics comparable with other diseases, clinicians and pathologists experience difficulties in its diagnosis.We aimed to reveal the clinical and histopathological characteristics of the retrospectively screened cases and to compare the prediagnoses and histopathological diagnoses of clinicians. Materials and methods: The data of the last 5 years were scanned and patients with histopathological diagnosis of CAT were included in the study. Results: A total of 65 patients, including 39 female and 26 male patients aged between 8 and 88, were included in the study. The female to male ratio was 1.5, and the mean age of the patients was 46.15 ± 21.8 years. The benign tumor rate was 95.4%, whereas the malignant tumor rate was 4.6%. 38.5% of the tumors were presenting sebaceous, 35.4% of them were presenting follicular, and 18.5% of them were presenting eccrine differentiation. It was most commonly seen in the head-neck region with a rate of 66.1%. When clinical and histopathological prediagnoses were compared, prediagnoses and histopathological diagnoses were compatible in 45% of the cases. Most frequently, it was the basal cell carcinoma, epidermal cyst, and sebaceous hyperplasia identified in preliminary diagnoses. Conclusion: Cutaneous adnexal tumors are very important, as they can accompany different syndromes and may be malignant. Due to difficulties in its clinical diagnosis, histopathological examination must be performed from suspicious lesions for definitive diagnosis.

Assessment of quality of life in Turkish children with psoriasis and their caregivers.

BACKGROUND/OBJECTIVES: The effect of pediatric psoriasis on quality of life has been demonstrated, but data regarding its influence on caregiver quality of life are scarce. The objective was to investigate how psoriasis affects quality of life of children and their caregivers. METHODS: This multicenter study included 129 children with psoriasis and their caregivers, who were family members accompanying patients to the clinic. Patient quality of life was measured using the Child Dermatology Life Quality Index. Caregiver quality of life was assessed using Dermatological Family Impact Scale, a 15-item questionnaire validated for use in the Turkish language. RESULTS: Mean Child Dermatology Life Quality Index score was 7.6, indicating a moderate effect on patient quality of life. Symptoms and feelings were the most severely impaired domains of patient quality of life, and emotions was the most severely impaired domain of caregiver quality of life. Dermatological Family Impact Scale score was significantly correlated with Child Dermatology Life Quality Index (correlation coefficient [r] = .554, P < .001) and Psoriasis Area and Severity Index (r = .350, P < .001). Caregivers of patients receiving systemic agents or phototherapy had relative impairment of multiple domains of quality of life compared to caregivers of patients receiving topical treatment only. CONCLUSION: Psychosocial effect of pediatric psoriasis was shown to extend beyond the individual, highlighting the importance of addressing patient and caregiver quality of life concerns in an integrated approach.

Subclinical left and right ventricular systolic dysfunction in Behcet's disease: a combined tissue doppler and velocity vector imaging study.

BACKGROUND: Behcet's disease (BD) is a chronic inflammatory disease characterized by recurrent oral and genital ulcerations and ocular lesions. Subclinical cardiac involvement may develop in BD patients. We aimed to evaluate subclinical left ventricular (LV) and right ventricular (RV) systolic dysfunction in BD patients without any apparent cardiovascular disease. METHODS: We studied 50 BD patients (43.8 ± 9.7 years, 59% men) and 30 healthy controls (45.4 ± 8.2 years, 60% men). Conventional echocardiography, tissue Doppler imaging, and velocity vector imaging-based strain measurements were performed to analyze LV and RV systolic functions. RESULTS: LV isovolumic myocardial acceleration, peak systolic velocity during isovolumic contraction (isovolumic contraction velocity), were significantly lower, while myocardial performance index was increased in BD patients. RV peak systolic velocity, isovolumic myocardial acceleration, and isovolumic contraction velocity were also markedly lower in BD patients. LV and RV longitudinal peak systolic strain and strain rate were significantly lower in patients than in controls, demonstrating subclinical ventricular systolic dysfunction. CONCLUSIONS: Ventricular long-axis functions are important markers of myocardial contractility. Novel echocardiographic techniques may provide additional data for detecting early deterioration in ventricular systolic function in patients with BD.

Evaluation of endothelial functions in patients with Behcet's disease without overt vascular involvement.

OBJECTIVES: Behcet's disease (BD) is a chronic inflammatory disease characterized by recurrent oral and genital ulcerations and ocular lesions. This multisystemic disorder primarily affects the vascular system. In the present study, we aimed to evaluate vascular endothelial function in patients with Behcet' s disease without vascular involvement. STUDY DESIGN: We studied 40 patients with BD (44.9±5.4 years) and 20 healthy controls (45.4±8.2 years). Brachial artery Doppler ultrasonography (USG) and bilateral carotid artery intima-media thickness measurements were performed. RESULTS: Basal diameter of the brachial artery were similar between the two groups. However, flow-mediated dilation was markedly impaired in patients with Behcet' s disease (p=0.03). Nitrate-induced dilation values were similar between the two groups (p=0.16). Carotid artery intima-media thickness was slightly increased in the patient group compared to the control group, but the difference did not reach statistical significance (0.69±0.15 to 0.59±0.09 respectively; p=0.06). CONCLUSION: Vascular endothelial function is impaired in BD. Brachial artery Doppler USG is a reliable and reproducible method to establish changes in vascular functions.