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.

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.

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.

Methyl(chloro)isothiazolinone contact allergy: a monocentric experience from Turkey.

INTRODUCTION: Due to their ubiquitous use, isothiazolinones caused allergic contact dermatitis epidemics and their use was restricted by legal regulations. OBJECTIVES: We aimed to evaluate demographic data, clinical findings, and patch test features of patients with methylisothiazolinone (MI) and/or methylchloroisothiazolinone(MCI) sensitivity in our study. METHODS: This study is a bidirectional and cross-sectional study, between July 2020 and September 2021. A total of 616 patients, including prospective and retrospective populations, demographic data, clinical findings, and patch test results were reviewed. Patients' demographics, patch test results, allergen sources, presence of occupational contact, and the characteristics of dermatitis attacks were recorded. RESULTS: A total of 50 patients with MI and MCI/MI sensitivity, 36 male (72%) and 14 female (28%), were included in our study. The overall prevalence of MI and MCI/MI between 2014 and 2021 was 8.4% (52/616) with two peak levels in 2015(21%) and 2021 (20%). A statistically significant relationship was found between shampoo use and facial involvement (p = 0.031), shower gel use and arm involvement (p = 0.027), wet wipe use and hand involvement (p = 0.049), detergent use and the pulps (p = 0.026) and the lateral aspects of fingers involvement (p = 0.048), water-based dye use and periungual involvement (p = 0.047). CONCLUSION: Although legal regulations related to MI and MCI/MI cause a decrease in the frequency of their sensitivities were still common causes of allergic contact dermatitis.

Relationship between surgical rhinoplasty and Demodex infestation: a case-control study.

Background/aim: Human Demodex mites are parasites that live in the pilosebaceous unit and can cause demodicosis. While demodicosis may occur as a primary skin disease, it may also result from immunosuppression and topical or systemic immunosuppressive therapies. Surgical rhinoplasty is one of the most commonly performed cosmetic procedures, and it is the cause of a variety of cutaneous complications, particularly acne, as it affects the skin's adnexal structures. Thus, this study aimed to investigate whether the cutaneous changes in surgical rhinoplasty patients render them vulnerable to Demodex infestation. Materials and methods: Individuals who had undergone rhinoplasty (patients) and age- and sex-matched healthy volunteers (controls) were included in this prospective case-control study. To determine the Demodex density, samples were collected from the malar and nasal regions of both the patients and controls using the standard superficial skin biopsy method. Results: A total of 50 rhinoplasty patients and 50 healthy controls were enrolled in the study. The Demodex density on the nose was significantly higher in the rhinoplasty patients (p = 0.0001). Furthermore, the frequency of xerosis and pustules was significantly higher in the rhinoplasty patients compared to the control group (p = 0.046 and p = 0.001, respectively). Conclusion: Surgical rhinoplasty may be a risk factor for demodicosis, and patients will recover faster after surgery with proper diagnosis and treatment.

Could there be an association between Hashimoto's thyroiditis and demodex infestation?

BACKGROUND: Human demodex mites are parasites that live in the pilosebaceous unit and can result in the disease demodicosis. While demodicosis may occur as a primary skin disease; immunosuppression, and topical or systemic immunosuppressive treatments can cause secondary demodicosis. It is known that thyroid hormones may cause skin changes, such as xerosis, and thereby may also modulate immune responses in the skin. OBJECTIVES: The aim of this study is to investigate whether or not that the changes occurring in the skin of patients with Hashimoto's Thyroiditis (HT) predispose to demodex infestation. METHODS: Seventy-eight patients being followed for a diagnosis of HT at Kocaeli University Endocrinology Outpatient Clinic, between January 2019 and March 2020, constituted the patient group. The control group consisted of 41 patients who did not have any chronic systemic or dermatological disease and were shown to have no thyroid disease by laboratory tests. Demodex intensity in the malar regions of the patient and control groups was determined using the standardized skin surface biopsy (SSSB) method and compared with each other. RESULTS: HT patients were significantly more likely to have increased demodex density and suggestive SSSB results than the controls (p < 0.001, p = 0.012, respectively). A significant correlation was found between demodex intensity and the findings of xerosis (p = 0.010, p = 0.011) and spiny follicular papules (p = 0.008, p = 0.008) in the patient or control groups, respectively. However, a significant correlation was identified between the demodex density and the symptoms of burning-stinging (p = 0.028), and feelings of dryness (p = 0.018) roughness (p = 0.028) only in the control group. CONCLUSION: Xerotic skin and/or impaired immune responses as a result of autoimmune changes in patients with HT may lead to secondary demodicosis.

Multicenter study evaluating the impact of COVID-19 outbreak on dermatology outpatients in Turkey.

COVID-19 pandemic has a significant impact on public health, whether directly or indirectly. The first case was seen in Turkey on March 11, and the World Health Organization (WHO) declared a pandemic on March 12, 2020. The study aimed to document the effect of pandemic on dermatology outpatient clinics in Turkey. Fifteen tertiary hospitals from 13 provinces were included in the study, which was conducted between January 12 and May 12, 2020. The International Codes of Diseases (ICD-10) categories and patients' characteristics were evaluated before and after the pandemic. A total of 164 878 patients, 133 131 before and 31 747 after the pandemic, were evaluated. The daily hospital applications were found reduced by 77%. The three of the most frequent diagnoses; dermatitis, acne, and psoriasis remained unchanged after the pandemic. While the frequency of herpes zoster, scabies, urticaria, pityriasis rosea and sexually transmitted diseases increased significantly; allergic and irritant contact dermatitis decreased after the pandemic. The applications regarding cutaneous neoplasms were considerably reduced during the pandemic, and this effect was more pronounced in cities with higher COVID incidence. The pandemic caused a noteworthy reduction in the number of patients accessing dermatological care. The pandemic caused significant changes in the frequency of a wide range of dermatological diseases. The application of cutaneous neoplasms is considerably reduced after the pandemic, and this effect was more pronounced in cities where pandemics are frequent. Therefore, the pandemic has resulted on numerous impacts on many critical issues in dermatology and dermatological care.

Imiquimod 5% Cream Versus Cryotherapy in Classic Kaposi Sarcoma.

BACKGROUND: Classic Kaposi sarcoma usually remains on the skin and has a slow progression; thus, local treatment methods are preferable. Imiquimod is an immunomodulatory agent with antiviral, antitumoural, and antiangiogenic properties that is expedient against Kaposi sarcoma. OBJECTIVES: We aimed to clarify whether imiquimod is effective on classic Kaposi sarcoma lesions by comparing imiquimod treatment with cryotherapy, which is the most-used treatment method in our department for this disease. METHOD: Patients with classic Kaposi sarcoma were included. All lesions of each patient were evaluated and measured by the blinded investigator considering infiltration and surface diameters. Then, lesions were categorized into 2 groups by the other investigator (nonblinded), and imiquimod 5% cream was administered 3 times per week without occlusion in 1 group. Cryotherapy was performed every 3 weeks in the other group. All lesions were reevaluated and measured at the end of 12 weeks by the blinded investigator. Initial and last measurements were compared between the treatment methods. RESULTS: Fifty lesions of 8 patients were included in this study. Imiquimod and cryotherapy were applied to 26 and 24 lesions, respectively. At the end of the study, statistically significant decreases were detected in all scores between weeks 0 and 12 with both treatment methods. Mean percentages of change in scores were not significantly different between the methods. CONCLUSIONS: Based on a limited number of patients and lesions treated, we believe imiquimod may be a suitable option to use for the treatment of classic Kaposi sarcoma.

Contact sensitivity to textile dyes in patients with pigmented purpuric dermatosis.

Purpose: The aetiology of pigmented purpuric dermatoses is unclear. Recent studies speculate about contact sensitivity to play a role in the aetiology of the disease. In most patients, the lesions begin and stay limited on the lower extremities as textile products are in tight contact with the skin. Also, textile dyes can cause contact dermatitis of purpuric type. This study was conducted in order to understand whether the contact sensitivity plays a critical role in the aetiology of pigmented purpuric dermatoses. Materials and methods: Patients with diagnosis of pigmented purpuric dermatoses were included in the study. Patch tests were applied at the back of all patients with textile series (Chemotechnique Diagnostics TF-1000) which have 33 allergens containing textile dyes, material and protectors by IQ Ultra Chamber. Test sites were evaluated at 48, 72, 96. h and on the 7th day according to the criteria of the International Contact Dermatitis Research Group. The evaluation results were recorded in patient files and the results were statistically compared. Results: Eighteen males (60%) and 12 females (40%), a total of 30 patients were included in the study. Schamberg disease in 23 patients (76.7%), lichen aureus in 5 patients (16.7%) and Majocchi disease in 2 patients (6.7%) were described out of total 30 patients. No contact sensitivity was detected in any of the patients. Conclusion: Contact sensitivity to textile dyes was not found as an etiologic factor in our study group.

A Rare Cause of Leg Ulcer: Calcinosis Cutis as a Part of CREST Syndrome.

Leg ulcers are not a disease themselves, they are a symptom of a disease. To manage them properly, finding the correct diagnosis of the disease is mandatory. Our case is a model to underline that leg ulcers are a significant burden for the patient, because leg ulcer got ahead of the patient's other serious complaints such as Raynaud's phenomenon or sclerodactyly. Furthermore, our patient is also a model, and an attentive clinical evaluation is inevitable to find the correct diagnosis. We present our case aiming to underline the significance of full dermatologic examination in each ulceration to discover underlying disease.

TNF-alpha inhibition prevents cognitive decline and maintains hippocampal BDNF levels in the unpredictable chronic mild stress rat model of depression.

Previous findings have shown that patients with depression express higher levels of proinflammatory cytokines such as TNF-α and IL-6. We have recently found that Infliximab (a TNF-α inhibitor) decreased anhedonia and despair-like behavior in the rat unpredictable chronic mild stress (UCMS) model of depression suggesting that inflammation might play an important role in depression. An increasing number of studies suggest that inflammation is also associated with cognitive impairments. The current study aimed to investigate the effect of UCMS on the cognitive performance of rats and their hippocampal BDNF levels and the effect of chronic Infliximab (5mg/kg/weekly, i.p.) treatment on these measures. Rats were subjected to different types of stressors daily for a period of 56 days to induce depression-like state. The UCMS resulted in impairments in spatial and emotional memory acquisition and retention with no effect on the level of locomotor activity. These behavioral effects of UCMS were accompanied by reduction in the level of BDNF in the CA1 and CA3 regions of the hippocampus. Chronic Infliximab treatment prevented the UCMS-induced cognitive impairments as well as the reduction in the levels of hippocampal brain-derived neurotrophic factor (BDNF). These results suggest that Infliximab improves the spatial and emotional memory impairments induced by chronic stress in rats likely through its effects on hippocampal function by modulating inflammation.

Blastic plasmacytoid dendritic cell neoplasm: skin and bone marrow infiltration of three cases and the review of the literature.

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a distinct and rare neoplastic entity and was classified as a subgroup of acute myeloblastic leukemia by the WHO in 2008. The median survival of patients was 15.2 months in a large case series. Allogeneic or autologous bone marrow transplantation has been recommended by some reports because of the disease's poor prognosis. We present three patients who presented with both skin and bone marrow infiltration. A 57-year-old man, a 62-year-old woman, a 64-year-old man were admitted to our outpatient clinic because of skin lesions. All of the patient's had bone marrow infiltration with positivity of the CD4, CD56, and CD123 staining. Survival of the patient's were 42, 6 and 12 months, respectively. Two of the patients who presented as blastic form didn't respond to any chemotherapy. BPDCN is a difficult disease to diagnosis and manage. CD4, CD56, CD123, CD303, and T cell leukemia/lymphoma 1. Cutaneous lesions can present as isolated nodules, macules, and disseminated macules and nodules. Positivities are crucial to the diagnosis of the disease in histological examination. Bone marrow infiltration or disease relapse at presentation were related to poor prognosis. Complete immunocytochemical staining must be performed for all patients who have cutaneous lesions with or without blood count abnormalities. Bone marrow (allogeneic or autologous) transplantation should be considered at the first remission.

Etanercept improves cognitive performance and increases eNOS and BDNF expression during experimental vascular dementia in streptozotocin-induced diabetes.

Diabetes mellitus (DM) is related to an increase in the incidence of vascular dementia. Inflammation is an important cause of endothelial dysfunction and cognitive deficits. The anti -tumor necrosis factor (TNF)-α fusion protein etanercept has been reported to exhibit memory-enhancing effects and endothelial protection. We tested the effect of etanercept on the cognitive endpoints and compared it with the cognitive dysfunction in streptozotocin (STZ )- induced DM rats by using the Morris water maze test (MWMT) and passive avoidance test (PAT). Systolic blood pressure (SBP), thoracic endothelial function, endothelial nitric oxide synthase (eNOS) expression, and hippocampal brain-derived neurotrophic factor (BDNF) expression were assessed. Thirty days after the induction of DM, rats exhibited severe learning and memory deficits associated with endothelial dysfunction and decreased expression of eNOS and BDNF. Chronic treatment with etanercept (0.8 mg/kg, s.c., every week for 30 days) improved cognitive performance, endothelial function, and expression of eNOS and BDNF in DM rats. Furthermore, the memory-improving effects of etanercept were independent of hyperglycemia. These data suggest that etanercept treatment prevents changes in endothelial function, eNOS expression, and hippocampal expression of BDNF and, consequently, vascular dementia in DM rats.

Proteus syndrome: report of a case with developmental glaucoma.

The purpose of this study was to report developmental glaucoma and pseudopapilledema in a patient with Proteus syndrome. We defined the presence of developmental glaucoma, right pseudopapilledema and myopia in a 4.5-year-old patient with Proteus syndrome. Marked right hemihypertrophy, lipoma, macrodactyly, and asymmetry of the limbs were observed on systemic examination. A cavernoma was also detected in magnetic resonance imaging of the brain. The patient underwent bilateral goniotomy surgery due to glaucoma. The surgical outcomes were satisfactory in both eyes. In conclusions developmental glaucoma and pseudopapilledema might be associated with Proteus syndrome.