Ultraviolet A1 Phototherapy in the Treatment of Early Mycosis Fungoides.

BACKGROUND: Phototherapy has been the mainstay of therapies for early mycosis fungoides (MF). The beneficial role of ultraviolet (UV) light on MF is suggested by the observation that lesions occur on non-sun-exposed areas. Therapeutic light sources that are available today are broadband UVB, psoralen and UVA, narrowband UVB, and long-wave UV (UVA1). Current literature provides increasing evidence on the use of UVA1 to treat MF. AIM: To investigate the treatment responses of early MF patients treated with fixed 30 J/cm2 doses of UVA1 phototherapy. MATERIAL AND METHODS: Nineteen patients with early MF, stage IA-IIA of the TNM staging system, received fixed 30 J/cm2 doses of UVA1, given 5 times weekly over 5 weeks. Therapeutic effectiveness was assessed by clinical examination and was confirmed by histological evaluation. RESULTS: Of the 19 patients, complete responses were achieved in 12 (63%) and partial responses were achieved in 7 (37%) patients after UVA1 radiation exposures. During the study, UVA1 therapy was well tolerated. During the follow-up, 7 (58%) of the 12 patients with complete response relapsed within 3 months of the UVA1 therapy. CONCLUSION: The current study provides clinical and histological evidence for the effectiveness of UVA1 (30 J/cm2 5 times a week for 5 weeks) as a skin-directed therapy in the treatment of early MF; however, such a treatment failed to maintain a long and sustained response. Thus, studies to identify the optimal dosing protocol regarding the therapeutic efficacy, the factors affecting relapse time/rate, and the necessity of maintenance treatment are needed.

Comparison of the efficacy of 308-nm excimer lamp monotherapy with topical tacrolimus or clobetasol 17-propionate combination therapies in localized vitiligo.

BACKGROUND: In recent years, excimer light treatments have come to the fore in localized vitiligo because of their high efficacy and safety. OBJECTIVE: We aimed to evaluate the efficacy of 308-nm excimer lamp as a monotherapy and its combination with tacrolimus 0.1% or clobetasol 17-propionate 0.05% ointment in localized vitiligo. METHODS: We performed a retrospective study including a total of 82 patients who were treated with excimer lamp monotherapy (EL group) (n = 30), excimer lamp + topical tacrolimus (EL + T group) (n = 29), or excimer lamp + topical clobetasol 17-propionate (EL + CS group) (n = 23). We used digital morphometric analysis for the evaluation of repigmentation. RESULTS: Median healing rate after 24 sessions was significantly higher in EL + T group (69%) than EL (42.5%) and EL + CS (44%) groups (P = 0.008 and P = 0.032). There were not any patients with face lesions in EL + CS group, so when these patients were excluded, EL + T and EL + CS groups had higher healing rates than EL group (P = 0.037 and P = 0.043). It was confirmed that combination with tacrolimus was superior to clobetasol-17 propionate (P = 0.048) with multivariate regression analysis. CONCLUSION: We noted that 308-nm excimer lamp therapy was efficacious; adding topical tacrolimus or topical clobetasol-17 propionate could increase response to treatment.

Evaluation of Clinicopathological Features and Associated Conditions in Erythema Annulare Centrifugum: A Retrospective Observational Analysis of 63 Patients.

INTRODUCTION: Erythema annulare centrifugum (EAC) is a rare reactive disease that typically presents as annular or polycyclic erythematous lesions. There are few studies about EAC in the literature; therefore, data on the pathogenesis of the disease are limited. OBJECTIVES: We aimed to examine the demographic, clinical, pathological characteristics and associated conditions of the patients with EAC. METHODS: We analyzed records of the patients with EAC who admitted to our clinic in the last four years retrospectively. RESULTS: A total of 63 patients, 39 women and 24 men, were included in our study. The mean age of the patients was 47.8±11.2 years. The mean disease duration was 15.54±12 months. The trunk and thigh were the most commonly involved sites (49%, 33%). Associated conditions were identified in 52.3% of the patients. Infections (N = 16), malignancies (N = 6), rheumatic diseases (n=4) and drugs (n=4) were the most common precipitating factors respectively. The most common infections were superficial fungal infections and Helicobacter pylori infection. Histopathologically, 10 patients had deep type and 53 patients had superficial type EAC. We determined that the disease duration from the onset of the disease was longer (12.3 versus 16.2 months) and the number of attacks (1.2 versus 2.8 attacks) was higher in the superficial type when compared to the deep type (P = 0.042, P = 0.038). CONCLUSIONS: In our study, infections, malignancies and rheumatic diseases were among the most common etiologic factors. We would also like to draw attention to Helicobacter pylori infection as a precipitating factor which may be a novel finding.

Oxidative Stress in Patients With Melasma: An Evaluation of the Correlation of the Thiol/Disulfide Homeostasis Parameters and Modified MASI Score.

Melasma is a common acquired hyperpigmentation disorder that affects mostly women and individuals with darker skin types. Oxidative stress may play a role in the pathogenesis of melasma. Dynamic thiol/disulfide homeostasis is one of the most important indicators of oxidative stress. This study aimed to investigate the presence of oxidative stress in patients with melasma by evaluating thiol/disulfide homeostasis. Sixty-seven patients with melasma and 41 healthy age- and sex-matched controls were included in the study. Disease severity was evaluated using the modified melasma area and severity index (mMASI). Thiol/disulfide homeostasis parameters of the melasma and control groups were measured using a novel, fully automated spectrophotometric method. Our data indicated the presence of oxidative stress in melasma, which may be correlated with disease severity. Because research on the presence of oxidative stress in melasma is limited, further studies are needed to support these conclusions.

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.

Analysis of clinical characteristics and factors affecting treatment responses in patients with pyoderma gangrenosum: a multicenter study of 239 patients☆.

BACKGROUND: Pyoderma Gangrenosum (PG) is a chronic disease characterized by recalcitrant skin ulcers. OBJECTIVE: We aimed to evaluate the demographic, clinical characteristics, treatments and factors affecting the treatment responses of patients with PG. METHODS: We performed a multicenter study of 12 tertiary care centers. We analyzed the data of the patients who were followed up with a diagnosis of PG between the years 2012‒2022 retrospectively. RESULTS: We included a total of 239 patients of whom 143 were female and 96 were male, with an average age of 54.2 ±â€¯17.4 years. The most common treatment was systemic steroids (n = 181, 75.7%). Among these patients, 50.8% (n = 92) used systemic steroids as the sole systemic agent, while 49.2% (n = 89) used at least one adjuvant immunosuppressive agent. The independent factors determined in regression analysis to influence response to systemic steroids positively were disease onset age ≥ 30-years, negative pathergy, absence of leukocytosis, negative wound culture, presence of a single lesion, and absence of upper extremity involvement. Biological agents were used in 18.4% (n = 44) of the patients in the present study. We also analyzed pathergy positive PG and early onset (onset age < 30) PG separately due to their distinct clinical features which were revealed during statistical analysis. STUDY LIMITATIONS: Retrospective nature of the present study. CONCLUSIONS: Analyses of the factors influencing treatment responses are addressed in this study. Also, we concluded that investigation for accompanying autoinflammatory diseases of pathergy positive PG and early onset PG is necessary and the patients in these two groups are more resistant to treatment, necessitating more complicated treatments.

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.

Evaluation of Oxidative Stress by Thiol/Disulphide Homeostasis in Patients with Mycosis Fungoides: A Prospective Double-Centre Study.

Background: Mycosis fungoides (MF) is the most common cutaneous T-cell lymphoma with unknown etiopathogenesis. Oxidant and antioxidant balance is important for cell function and normal metabolism. An imbalance between pro-oxidants and antioxidants causes oxidative stress. A recent focus has been on thiol/disulphide homeostasis as a novel marker of oxidative stress. Aims and Objectives: This study aimed to evaluate the role of oxidative stress in MF by analysing thiol/disulphide homeostasis. Materials and Methods: A total of 103 patients (48 female, 55 male) and a control group of 120 healthy individuals (48 female, 72 male) from two tertiary care hospitals were included in our study. Serum native thiol, total thiol and disulphide levels were evaluated using novel method developed by Erel and Neeliolu. Results: Native thiol levels were 340.30 ± 87.44 in the patient group and 401.62 ± 69.45 in the control group. Total thiol value was 374.17 ± 87.78 in the patient group and 428.54 ± 70.05 in the control group. Native thiol and total thiol levels were significantly lower in the patient group compared to the control group (P < 0.001 and P < 0.001). The disulphide value was 16.93 ± 6.46 in the patient group and 13.46 ± 5.06 in the control group. Disulphide levels were found to be significantly higher in the patient group compared to the control group (P < 0.001). Conclusions: In our study, thiol/disulphide balance shifted towards disulphide which indicates the presence of oxidative stress especially in the early stage while 93.2% of our patients had early-stage MF. We think that this may have pathogenetic and prognostic significance.

Prevalence of smoking, alcohol consumption and metabolic syndrome in patients with psoriasis.

BACKGROUND: Coexistence of obesity, hypertension, insulin resistance and dyslipidemia is defined as metabolic syndrome (MBS), which is among the important risk indicators for cardiovascular diseases, diabetes and stroke. Smoking and alcohol consumption are the other factors which lead to an increase in the risk of cardiovascular disease. OBJECTIVE: To investigate the prevalence of metabolic syndrome, smoking and alcohol consumption in psoriasis patients and the relationship between disease severity and these factors. METHODS: This cross-sectional study enrolled 563 patients with chronic plaque-type psoriasis, all of which completed a questionnaire and underwent a complete physical examination. Data about MBS components, psoriasis severity/duration, smoking and alcohol consumption, and cardiovascular diseases were recorded. RESULTS: A total of 563 patients with ages ranging from 18 to 78 years were evaluated. Metabolic syndrome was found in 12.6% of the patients [central obesity (38.7%), hypertension (14.3%), dyslipidemia (18.6%), diabetes (9.2%)], while 50.3% had smoking, and 3.3% had alcohol consumption. Patients with metabolic syndrome were older and more likely to have a longer disease duration than those without metabolic syndrome (p<0.05 for each). The prevalence of metabolic syndrome was higher in women than in men. Psoriasis was more severe in patients with central obesity, diabetes and smoking than in those without (p<0.05 for each). STUDY LIMITATIONS: Retrospective design. CONCLUSIONS: Our results indicate that MBS is a risk factor for psoriasis patients with advanced age. The relationship between disease severity and obesity, diabetes, and smoking in psoriasis patients indicates that the patients should be informed about the potential metabolic risks and receive therapies for behavioral changes besides anti-psoriatic treatment in order to minimize these risks.

Excellent response to tofacitinib treatment in a patient with alopecia universalis.

Alopecia universalis (AU) is generally considered a variant of alopecia areata (AA), in which the treatment options seldom provide satisfactory results. However, successful treatment of several cases of AA and its variants with oral Janus kinase (JAK) inhibitors have been reported recently. Here we report a 23-year-old female patient with AU successfully treated with tofacitinib, a selective JAK-3 inhibitor. The initial tofacitinib dose was 5 mg twice daily. After 2 months of treatment, partial hair regrowth was seen on the scalp and eyebrows. Thereafter, the dose was increased to 10 mg in the morning and 5 mg at night. By 6 months of the treatment, there was complete hair regrowth throughout the entire body. Our patient tolerated tofacitinib well, without any significant side effects. Tofacitinib emerges as a promising novel therapy in alopecia universalis. We believe further study is required to establish the safety and confirm the efficacy of tofacitinib treatment for alopecia universalis.

Pyoderma Gangrenosum: A Report of 27 Patients.

The aim of this study was to document the profile of pyoderma gangrenosum (PG) patients who were treated in our clinic and to compare our patients' clinical and demographic characteristics with those described in the literature to help our understanding of the total burden of PG. A total of 27 (17 women, 10 men) patients with a mean age 48.6 years at diagnosis were included in the study. Seven (25.9%) of them had 3 or more ulcers. The lower extremity was the most common site of occurrence (92.5%). During the study period, 6 patients were admitted twice, and 7 were admitted 3 or more times (range 1 to >10), and the median length of stay was 27 days. A concomitant disease was present in PG patients especially in those between 20 and 40 years of age. Systemic therapy was used in 21 (91.3%) patients, 17 patients were treated with systemic corticosteroids, either alone in 7 patients or combination with other agents in 10 patients. Three of our patients died because of disease or treatment-related complications during the study period. Despite the high (70.3%) percentage of the PG patients with improving disease at the time of discharge, 1 year after hospital discharge, one third of our 27 patients still had PG requiring readmission. In conclusion, none of the current therapies provide satisfying results in all of the patients, and our data indicate a long-standing unmet need for effective therapy for the unexpected course of PG.

A Rare Cause of Lower Extremity Ulcers: Prolidase Deficiency.

Prolidase deficiency is an autosomal recessive disorder, which is associated with chronic skin ulcers, a characteristic facial appearance, mental retardation, and recurrent infections. This study describes 4 patients with recurrent leg ulcerations and abnormal facies who were first clinically suspected of prolidase deficiency and then biochemically confirmed. Two siblings and 2 other patients were admitted to our clinic at different times, and they had some common features such as chronic leg and foot ulcers recalcitrant to treatment, consanguineous parents, facial dysmorphism, mental retardation, and widespread telangiectasias. Physical examination and detection of low prolidase level in blood finally led us to the diagnose of ulcers secondary to prolidase deficiency. Prolidase deficiency is a rare genodermatosis and must be considered in the differential diagnosis of recurrent leg and foot ulcers that develop at an early age.

Prevalence of smoking, alcohol consumption and metabolic syndrome in patients with psoriasis

Abstract: Background: Coexistence of obesity, hypertension, insulin resistance and dyslipidemia is defined as metabolic syndrome (MBS), which is among the important risk indicators for cardiovascular diseases, diabetes and stroke. Smoking and alcohol consumption are the other factors which lead to an increase in the risk of cardiovascular disease. Objective: To investigate the prevalence of metabolic syndrome, smoking and alcohol consumption in psoriasis patients and the relationship between disease severity and these factors. Methods: This cross-sectional study enrolled 563 patients with chronic plaque-type psoriasis, all of which completed a questionnaire and underwent a complete physical examination. Data about MBS components, psoriasis severity/duration, smoking and alcohol consumption, and cardiovascular diseases were recorded. Results: A total of 563 patients with ages ranging from 18 to 78 years were evaluated. Metabolic syndrome was found in 12.6% of the patients [central obesity (38.7%), hypertension (14.3%), dyslipidemia (18.6%), diabetes (9.2%)], while 50.3% had smoking, and 3.3% had alcohol consumption. Patients with metabolic syndrome were older and more likely to have a longer disease duration than those without metabolic syndrome (p<0.05 for each). The prevalence of metabolic syndrome was higher in women than in men. Psoriasis was more severe in patients with central obesity, diabetes and smoking than in those without (p<0.05 for each). Study Limitations: Retrospective design. Conclusions: Our results indicate that MBS is a risk factor for psoriasis patients with advanced age. The relationship between disease severity and obesity, diabetes, and smoking in psoriasis patients indicates that the patients should be informed about the potential metabolic risks and receive therapies for behavioral changes besides anti-psoriatic treatment in order to minimize these risks.