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 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.

Acquired Perforating Dermatosis: Clinical and Histopathological Analysis of 95 Patients From One Center.

INTRODUCTION: Acquired perforating dermatosis (APD) is a disease group characterized by transepidermal elimination of dermal connective tissue materials such as collagen, elastic fibers, and keratin through the epidermis and observed with pruritic skin lesions. OBJECTIVES: In this study, we aim to clarify the clinical, histopathological, and dermoscopic characteristics of APD, identify the associated systemic disease, and figure out treatment options. METHODS: This study was designed as a single-center retrospective, observational, cross-sectional study. We evaluated all accessible APD cases between January 2004 and June 2022 in a tertiary care hospital. RESULTS: A total of 95 patients with confirmed APD were included in the study. Sixty percent of the patients were women and 40% were men. The median age at diagnosis was 63.1 years (35-85 years). The most common site of lesions was the lower extremities which were detected in 86.31% of the patients. The concomitant systemic disease was identified in 84.21% of the patients. The most common systemic disease was type 2 diabetes mellitus (65.26%). Antihistamines and topical corticosteroids were the most commonly prescribed treatment agents. CONCLUSIONS: Transepidermal elimination of dermal connective tissue components is a feature of APD and the disease usually presents with pruritic papules and nodules with central keratotic crust or plug. The diagnosis of APD requires a clinical examination and histological investigation. APD is usually accompanied by systemic comorbidities. There are several topical and systemic medications available for APD, however, sometimes the therapy might be challenging.

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.

Postoperative Pyoderma Gangrenosum Following Carpal Tunnel Surgery: A Case Report and Review of the Literature.

Pyoderma gangrenosum (PG) is a neutrophilic dermatosis characterized by painful ulcerated lesions. Postoperative PG, which typically begins with erythema and severe pain within two weeks after surgery, progresses into ulcerated lesions. It is often misdiagnosed as it resembles necrotizing skin infections, resulting in delayed treatment. Cases of postoperative PG located in the upper extremity are uncommon. In this case report, we discuss a male patient who developed postoperative PG after carpal tunnel surgery.

Clinical, histopathological and immunohistochemical evaluation of ultraviolet A1 treatment in early-stage mycosis fungoides.

BACKGROUND/PURPOSE: Mycosis fungoides (MF) is the most common variant of cutaneous T-cell lymphomas primarily involving the skin. Early-stage MF is characterised by non-specific skin lesions and non-diagnostic biopsies. While skin-focused treatments, such as PUVA and narrowband UVB (nbUVB), are the most frequently recommended treatments, the UVA1 efficacy has been researched in recent years. The purpose of this study was to evaluate the clinical, histopathological and immunohistochemical aspects of UVA1 treatment in patients with early-stage MF. METHODS: The modified severity weighted assessment scale (mSWAT) was used for total skin body scoring before and after treatment. Skin punch biopsies were taken from the patients before and after treatment. UVA1 therapy was performed five times each week. RESULTS: This study included 26 patients with early-stage MF. The total number of UVA1 sessions varied between 15 and 34. Complete response was observed in 8 (30.8%) of 26 patients (30.8%). The median mSWAT score decreased statistically significantly from 7.1 to 2.0 after treatment (p < .001). Histopathological complete response was observed in 2 (9.5%) of 21 patients. A statistically significant decrease in dermal interstitial infiltrate was observed on histopathological examination after treatment (p = .039). Epidermal CD4/CD8 levels decreased statistically significantly higher from a median of 2.5-1.2 in the complete clinical response group after treatment (p = .043). CONCLUSION: According to our results, UVA1 treatment has an effect on early-stage MF in terms of clinical, histopathological and immunohistochemistry.

Novel Compound Heterozygous Mutations of TGM1 Gene Identified in a Turkish Collodion Baby Diagnosed with Non-Bullous Congenital Ichthyosiform Erythroderma.

Autosomal recessive congenital ichthyosis (ARCI) is a group of diseases presenting as collodion baby at birth. ARCI is categorized as Harlequin ichthyosis, lamellar ichthyosis, and non-bullous congenital ichthyosiform erythroderma (NBCIE), bathing suit icthyosis (BSI) and others. We describe the case of a male newborn with NBCIE whose whole exome sequencing revealed two variants of TGM1 gene (NM_000359.3) in a compound heterozygous state: c.790C>T (p.Arg264Trp) in exon 5 and c.2060G>A (p.Arg687His) in exon 13. In the literature, the Arg264Trp variant has been reported as homozygous or compound heterozygous with other variants in patients with BSI. In contrast, the Arg687His variant has been reported only as homozygous in patients with BSI. To the best of our knowledge, this is the first case whose two compound heterozygous variants, exhibiting the NBCIE phenotype, instead of the BSI.

Could serum HMGB1 levels be a predictor associated with psoriatic arthritis?

Background/aim: Psoriasis is a chronic autoimmune disease that predominantly affects the skin and musculoskeletal system. We hypothesized that HMGB1, an inflammatory nuclear protein, may play a role in the musculoskeletal involvement of psoriasis. Methods: Forty patients with psoriasis and 45 with psoriatic arthritis were involved in the study; the results were compared with 22 healthy controls. Serum HMGB1 levels were evaluated from peripheral blood samples. Results: Serum HMGB1 levels were found to be significantly higher in patients with psoriasis regardless of joint involvement (p < 0.001). Also, HMGB1 levels were correlated with the extent of psoriasis. Conclusion: Serum HMGB1 levels may contribute to the progression of psoriasis to psoriatic arthritis and correlate with the severity of skin involvement.

Psoriasis is an autoimmune skin disease that may also affect the joints. Factors leading to the progression of psoriasis to psoriatic arthritis are still a mystery despite an increasing number of animal studies and real-life data. HMGB1 is a nuclear protein that leads to an increase in molecules that increase inflammation (TNF-α, IL-1 and IL-6) in the body. Until now, there was no report about the relationship between psoriatic arthritis and serum HMGB1 levels. Our study aimed to find any difference in HMGB1 levels between healthy and psoriatic patients. Psoriatic arthritis patients had higher levels of serum HMGB1 than patients with psoriasis. Also, HMGB1 levels were correlated with the severity of skin involvement. Our results showed that serum HMGB1 may indicate a high risk for developing psoriasis that involves the joints. Therefore the HMGB1 level in psoriasis patients can potentially serve as a predictor associated with disease severity and the risk of developing psoriatic arthritis.

Topical Insulin Application in the Management of Resistant Leg Ulcers in a Patient With Prolidase Deficiency: A Case Report.

Leg ulcers are a significant cause of morbidity and mortality and can be caused by vascular, neuropathic, infectious, and traumatic factors, as well as rare metabolic diseases like prolidase deficiency. Despite various wound care methods and systemic treatments, managing ulcers can be challenging. This case presents a male patient with prolidase deficiency for 35 years whose leg ulcers were resistant to standard treatments such as wound dressings, topical treatments, and hyperbaric oxygen therapy. Considering the ulcers' resistant nature, we applied topical insulin to ulcers as an add-on therapy and observed clinical improvement. In this case, we want to emphasize the potential of insulin as a supplementary treatment agent in prolidase deficiency-induced ulcer treatment.

Use of Topical Insulin in the Wound Healing Process After Ultrasonography-Guided Modified Deroofing Surgery in a Hidradenitis Suppurativa Patient: A Case Report.

Hidradenitis suppurativa (HS) is an inflammatory disease causing nodules, abscesses, sinus tracts, and scars in fold areas. It significantly impacts patients' quality of life. Surgical treatments are becoming popular in dermatology, with deroofing being a common procedure. However, recurrence rates can be high due to not removing fibrotic areas and scar tissue entirely. Recent efforts have focused on removing these tissues to achieve lower recurrence rates. This case report describes a male patient with HS who underwent a newly defined ultrasonography-guided modified deroofing surgery for HS. The wound-healing process was then accelerated with the application of topical insulin. In this case, we would like to highlight the significance of using ultrasonography before HS surgery, confirm the importance of modified deroofing surgery, and emphasize that insulin can be used as an effective supplementary treatment for ulcer management.

The Clinical Characteristics of Patients With Pyoderma Gangrenosum in a Tertiary Referral Hospital: A Retrospective Cohort Study.

Since pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis, epidemiological and clinical data on the disease are scarce. In this single-center retrospective study, we aim to evaluate the clinical characteristics, underlying systemic associations and treatment modalities in patients with PG in a university hospital between 2014 and 2022. It is known that PG most commonly affects the lower extremities, but extracutaneous involvement should also be kept in mind. PG is usually associated with various comorbidities that share a similar inflammatory pathogenesis with the disease. The prevalence of PG-related comorbidities varies in different studies, arthritis and solid organ malignancies were observed most frequently in the current study. Non-PG-related comorbidities including diabetes mellitus, hypertension and peripheral vascular disease can adversely affect wound healing and limit treatment options; therefore, a holistic approach to patients with PG is crucial. Consistent with literature, the mainstay of treatment for PG is systemic corticosteroids and cyclosporine. However, the implementation of biologic agents in treatment-resistant patients is an increasingly important issue in the literature. Antitumor necrosis factors (anti-TNFs) are the most commonly preferred biological therapies, and these agents seem to have paved the way for a paradigm shift in the treatment of PG. In the present study, a relatively high per cent of (23.3%) patients treated with anti-TNFs, most commonly infliximab (87.5%). Recurrence was observed in 46.7% of our patients in the follow-up period and the relapse rate was found to be higher in patients using multiple systemic agents compared to those using single agents (64.7% vs 23.1%, P < .05). In conclusion, we emphasize that early diagnosis and treatment by considering the patient's comorbidities are important in preventing complications, and biologic treatments seem particularly promising in treatment-resistant patients.

Topical Insulin as an Add-On Therapy for Leg Ulcer: A Case Report.

Leg ulcers affect millions of people worldwide, and are a significant cause of morbidity and mortality. Various factors can be etiological agents of leg ulcers such as vascular, neuropathic, infectious, and traumatic factors. Treatment of the leg ulcer can be difficult in some cases despite using different systemic treatments and local wound care, but various newly defined treatment modalities are discussed in the literature and topical insulin application is one of them. Insulin is a hormone that is essential for regulating blood glucose and lipid levels, also insulin can have local effects when applied topically. Various mechanisms like regulation of inflammation, collagen synthesis, and angiogenesis have been explained to understand topical insulin's effects on the wound. There are case reports and studies on the usage of topical insulin on diabetic ulcers and decubitus ulcers. We applied topical insulin as an add-on therapy on a treatment-resistant leg ulcer and observed the healing of the lesion. The use of topical insulin as an add-on therapy may reduce treatment time and speed up wound healing. Topical insulin can be considered as an additional therapy for treatment-resistant ulcers.

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.