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Livedoid vasculopathy (LV) is a rare, chronic, and occlusive disease of the veins supplying the upper parts of the skin. The pathogenesis of the disease is not precisely understood, and its attacks are often unpredictable but tend to worsen during the summer. LV affects women more often. This increased risk for LV in women might be related to sex-specific physiological conditions, such as pregnancy, or a higher incidence of LV-associated conditions, such as connective tissue diseases, hypercoagulable states, and venous stasis in women. The typical clinical appearance of LV consists of three main findings: livedo racemose, atrophie blanche, and skin ulcers. The purpose of this comprehensive review was to analyze LV in all aspects and mainly focus on early diagnosis for successful clinical management with a holistic and multidisciplinary approach. A detailed history, dermatological examination, and laboratory testing are essential for a diagnosis of LV. When LV is clinically suspected, a skin biopsy should be taken to confirm the diagnosis. Another critical step is to investigate the underlying associated conditions, such as connective tissue diseases, hypercoagulable states, thrombophilia, and malignancy. Unfortunately, no associated conditions can be detected in approximately 20% of all cases (idiopathic LV) despite all efforts. The diagnosis of the disease is delayed in most patients. Thus, irreversible, permanent scars appear. Early and appropriate treatment reduces pain and prevents the development of scars and other complications. Antiplatelet drugs and anticoagulants can be preferred as the first-line treatments along with general supportive measures. Other therapeutic options might be considered in unresponsive cases. Preference for refractory cases is based on availability, clinical experience, and patient-related factors (comorbidities, age, sex, and compliance). These include anabolic steroids, intravenous immunoglobulin, hyperbaric oxygen therapy, psoralen-ultraviolet A, vasodilators, fibrinolytics, immunomodulators, and immunosuppressives.

BACKGROUND Skincare products and cosmetic procedures are used as an adjunct or complementary to conventional drug therapy for acne vulgaris (AV). OBJECTIVE To evaluate the use of skincare products and the frequency of cosmetic procedures in AV treatment. METHODS A total of 1,755 patients with AV completed the survey prepared by the researchers and the Cardiff Acne Disability Index (CADI) questionnaire. The clinical findings and the Food and Drug Administration (FDA) severity scores were recorded by the dermatologists. RESULTS For AV, 66.7% of the patients stated that they used skincare products and 26.7% had undergone cosmetic procedures. The use of skincare products was statistically significantly higher in women (female: 74.5%, male: 57.7%, p

Background Palmoplantar pustulosis (PPP) is a rare, chronic, inflammatory skin disease characterized by sterile pustules on palmar or plantar areas. Data on PPP are scarce. Aim To investigate the clinical characteristics and risk factors for disease severity in a large cohort of Turkish patients with PPP. Methods We conducted a cross-sectional, multicentre study of patients with PPP recruited from 21 tertiary centres across Turkey. Results In total, 263 patients (165 women, 98 men) were evaluated. Most patients (75.6%) were former or current smokers. The mean Palmoplantar Pustulosis Area and Severity Index (PPPASI) was 8.70 +/- 8.06 and the mean Dermatology Life Quality Index (DLQI) score was 6.87 +/- 6.08, and these scores were significantly correlated (r = 0.52, P < 0.001). Regression analysis showed that current smoking was significantly associated with increased PPPASI (P = 0.03). Coexisting psoriasis vulgaris (PsV) was reported by 70 (26.6%) patients. Male sex prevalence, PPP onset incidence, disease duration, DLQI, and prevalence of nail involvement and psoriatic arthritis (PsA) were significantly increased among patients with PPP with PsV. Of the 263 patients, 18 (6.8%) had paradoxical PPP induced by biologic therapy, and these patients had significantly increased mean DLQI and prevalence of PsA (r = 0.03, P = 0.001). Conclusion Our data suggest that smoking is a risk factor for both PPP development and disease severity. Patients with PPP with PsV present distinct clinical features and patients with biologic therapy-induced paradoxical PPP have reduced quality of life and are more likely to have PsA.

Objective: The aim of this multi-center study was to assess predictive factors for work-day loss as an indirect cost element in Behçet's syndrome (BS). Methods: In this cross-sectional, multi-center study, 834 BS patients (F/M: 441/393, age mean: 38.4 ± 10.9 years) were included. Data were collected by a questionnaire regarding treatment protocols, disease duration, smoking pattern, frequency of medical visits during the previous year and self-reported work-day loss during the previous year. Results: Work-day loss was observed in 16.2% of patients (M/F: 103/32). The percentages of being a smoker (81.8%), using immunosuppressive (IS) medications (82%), and having disease duration

© 2021 Taylor & Francis Group, LLC.Background: Immunosuppressive therapy has been a great concern during the pandemic. This study aimed to evaluate the pandemic's impact on psoriasis patients treated with immunosuppressive drugs. Material and Methods: The multicenter study was conducted in 14 tertiary dermatology centers. Demographic data, treatment status, disease course, and cases of COVID-19 were evaluated in patients with psoriasis using the immunosuppressive treatment. Results: Of 1827 patients included, the drug adherence rate was 68.2%. Those receiving anti-interleukin (anti-IL) drugs were more likely to continue treatment than patients receiving conventional drugs (OR = 1.50, 95% CI, 1.181–1.895, p =.001). Disease worsening rate was 24.2% and drug dose reduction increased this rate 3.26 and drug withdrawal 8.71 times. Receiving anti-TNF or anti-IL drugs was associated with less disease worsening compared to conventional drugs (p =.038, p =.032; respectively). Drug withdrawal causes were ‘unable to come’ (39.6%), ‘COVID concern’ (25.3%), and ‘physician’s and patient’s co-decision’ (17.4%). Four patients had COVID-19 infection with mild symptoms. The incidence was 0.0022% while it was 0.0025% in the general population. Conclusion: Our study shows that psoriasis patients using systemic immunosuppressive do not have a higher, but even lower COVID-19 risk than the general population, and treatment compliance with biological drugs is higher.

Behçet's disease (BD) is a chronic, relapsing inflammatory, multisystem disease of unknown etiology. The disease has a wide clinical spectrum of mucocutaneous lesions and ocular, vascular, articular, neurologic, gastrointestinal and cardiac involvement. Although the number of effective drugs used in the disease's treatment has increased in recent years, BD is still associated with severe morbidity because of mainly mucocutaneous, articular and ocular symptoms and an increased mortality because of large vessel, neurological, gastrointestinal and cardiac involvement. Many factors are associated with a more serious course, such as male gender and a younger age of onset. While the severity of the disease is more pronounced in the first years of the disease, it decreases in most patients after the age of forties. The primary goal of treatment should be the prevention of irreversible organ damage. Therefore, early diagnosis and appropriate treatment and close follow-up are mandatory to reduce the morbidity and mortality of the disease. Treatment varies depending on the organ involved and the severity of the involvement. For all these reasons, the treatment should be personalized and arranged with a multidisciplinary approach according to the organs involved. Treatment is mainly based on suppression of the inflammatory attacks of the disease using local and systemic immunomodulatory and immunosuppressive drugs. In this review, based on the mainly controlled studies and personal experience in clinical practice and basic research in this field, we propose a stepwise, symptom-based, algorithmic approach for the management of BD with a holistic perspective.

Behçet's disease (BD) is a chronic, relapsing, systemic inflammatory disease with clinical features showing mucocutaneous lesions involving the ocular, articular, and further miscellaneous organs. Mucocutaneous manifestations, one of the most characteristic signs of BD, have been most commonly observed upon onset or at any disease stage and are exceptionally important in its diagnosis. Given the lack of specific diagnostic laboratory tests for BD, diagnosis has been based on clinical findings. All diagnostic criteria published have thus far relied heavily on mucocutaneous manifestations, particularly oral ulcers (OU), genital ulcers (GU), cutaneous lesions, and pathergy test positivity. Worldwide, OU, GU, cutaneous lesions, and ocular and articular manifestations have been the most common symptoms, with erythema nodosum (EN)-like lesions and papulopustular lesions being the most prevalent cutaneous manifestations. While majority of the patients worldwide have reported OU as the most frequent symptom upon disease onset, GU, and EN-like lesions have also been identified upon onset. Considering that mucocutaneous symptoms precede severe organ involvement in most patients, familiarity with such symptoms is imperative for early diagnosis and prevention of potentially serious organ involvement through appropriate management.

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. 15 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 164878 patients, 133131 before and 31747 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. This article is protected by copyright. All rights reserved.

Background: Hidradenitis suppurativa (HS) is a chronic, relapsing and debilitating inflammatory disease associated with profound morbidity. Aim: In this multicentre study, we investigated the demographic and clinical features of HS, and determined risk factors of disease severity. Methods: In total, 1221 patients diagnosed with HS from 29 centres were enrolled, and the medical records of each patient were reviewed. Results: The mean age of disease onset was 26.2 ± 10.4 years, and almost 70% (n = 849) of patients were current or former smokers. Mean disease duration was 8.9 ± 8.4 years with a delay in diagnosis of 5.8 ± 3.91 years. Just over a fifth (21%; n = 256) of patients had a family history of HS. The axillary, genital and neck regions were more frequently affected in men than in women, and the inframammary region was more frequently affected in women than in men (P

Background: Internalized stigma, adoption of negative attitudes and stereotypes of the society regarding persons' illness, has not been studied previously in pediatric psoriasis patients. Objective: We aimed to investigate the internalized stigma in pediatric psoriasis patients and to determine differences according to factors affecting internalized stigma compared to adult psoriasis patients. Methods: This multicenter, cross-sectional, comparative study included 125 pediatric (55 female, 70 male; mean age±standard deviation [SD], 14.59±2.87 years) and 1,235 adult psoriasis patients (577 female, 658 male; mean age±SD, 43.3±13.7 years). Psoriasis Internalized Stigma Scale (PISS), Dermatology Life Quality Index (DLQI), Perceived Health Status (PHS), and the General Health Questionnaire (GHQ)-12 were the scales used in the study. Results: The mean PISS was 58.48±14.9 in pediatric group. When PISS subscales of groups were compared, the pediatric group had significantly higher stigma resistance (p=0.01) whereas adult group had higher scores of alienation (p=0.01) and stereotype endorsement (p=0.04). There was a strong correlation between mean values of PISS and DLQI (r=0.423, p=0.001). High internalized stigma scores had no relation to either the severity or localization of disease in pediatric group. However, poor PHS (p=0.007) and low-income levels (p=0.03) in both groups, and body mass index (r=0.181, p=0.04) in the pediatric group were related to high PISS scores. Conclusion: Internalized stigma in pediatric patients is as high as adults and is related to poor quality of life, general health, and psychological illnesses. Unlike adults, internalized stigma was mainly determined by psoriasis per se, rather than disease severity or involvement of visible body parts, genitalia or folds. Copyright © The Korean Dermatological Association and The Korean Society for Investigative Dermatology.

The aim of the present study was to examine the effects of age on mucocutaneous activity by using moderation analysis in Behçet’s syndrome (BS). In this cross-sectional study, 887 BS patients (female : male, 481:406; mean age, 38.4 ± 10.9 years) followed in 13 tertiary centers in Turkey were included. Mucocutaneous activity was evaluated by using the Mucocutaneous Index (MI) according to sex and disease course. Moderation analysis was performed to test the effect of age on mucocutaneous activity. A moderator variable is a third variable and affects the relationship between independent and outcome variables. Age was chosen as a potential moderator variable (interaction effect), MI score as the outcome variable and sex as an independent variable in the analysis. The moderation analysis tested the effects of age in three steps: whole BS patient group, patients without systemic involvement and those with systemic involvement. The moderation model was only significant in BS patients with systemic involvement (P = 0.0351), and a significant relationship was observed between female sex and MI score (P = 0.0156). In addition, the interaction plot showed that female patients had increased MI scores compared with male patients, especially in the 28-year-old age group (P = 0.0067). Moreover, major organ involvement was newly diagnosed in the majority of these young female BS patients. Our results suggest that the relationship between sex and mucocutaneous activity was moderated by age in the systemic involvement group. Also, increased mucocutaneous activity may be associated with new major organ involvement in young female BS patients with systemic involvement. © 2020 Japanese Dermatological Association

Scabies outbreaks were reported in many countries especially from Africa, but had not been reported in Turkey before.1 This is a multicentre study with compiling the data of patients with scabies in 12 tertiary level dermatology clinics located in distinct geographical regions of Turkey. The medical ethical committee of Ondokuz Mayis University Medical Faculty approved the study.

Objective:The aim of this study is to determine chronologically the occurrence of clinical forms and individual lesions of psoriasis patients. Methods:A total of 100 patients (mean age 42.07±16.12 years) were included in this retrospective cohort study. Individual lesions and changes in clinic forms recorded chronologically. The patients were also assessed for accompanying comorbidities during the course of disease. Results:Skin manifestations were the most frequent observed onset area of the disease (95%) and the most common clinical morphology at the onset of the disease was psoriasis vulgaris (92%). Scalp (19%) was the most frequent onset area of the disease. Clinical morphologic changes were detected in 26 patients during the course of the disease. The most common clinical morphological change was guttate psoriasis (n=17). In survival analysis, average time between the appearance of the first symptom and skin involvement, nail involvement, and articular involvement was calculated as 0.23±0.106, 5.89±1.07 and 2.25±1.10 (mean ± standard error) years, respectively. During the average 11-year disease course, two-fold increase observed in accompanying diseases. Conclusion:Our study shows that psoriasis starts with the skin involvement, nail and joint involvement develop later. Psoriasis vulgaris is the most frequently observed clinical morphology; scalp is the most commonly affected skin area at the onset of the disease. Over time, the number of comorbidities accompanying psoriasis increases.

Objective: Sensorineural hearing loss can occur as a complication of autoimmune and inflammatory diseases. Although psoriasis is also a chronic inflammatory skin disease characterized by T-cell mediated hyper proliferation of the keratinocytes, the information about the relationship between audiological disorders is limited in the literature and the relationship with vestibular disorders has not been investigated before. In this study, we aimed to investigate the presence of audiovestibular disorders and their relationship with disease parameters. Methods: Sixty-one patients with psoriasis and 61 healthy individuals were included in this prospective cross-sectional study. Those with possible etiologic factors that may lead to hearing and balance disorders were not included in the study. All participants were first performed a full ear, nose and throat examination. Subsequently, full audiological examination (pure audiometry, autoacoustic emission, stapes reflex, detection threshold of speech and discrimination) and electronystagmography tests were performed in the audiology laboratory where sound isolation was provided. Psoriasis severity was assessed by psoriasis area and severity index, body surface area and general evaluation of researcher. Results: There were significant differences between patients and controls in terms of audiovestibular symptoms. According to audiograms, predominant bilateral sensorineural hearing loss was detected in high frequency in psoriasis patients. The vestibular abnormalities in patients with psoriasis were found to be more frequent than those in controls, only saccadic test values were observed as statistically significant. Conclusion: Our study demonstrates that audiovestibular abnormalities are significantly associated with psoriasis. Therefore, patients with psoriasis should be evaluated for the co-occurrence of hearing loss or vestibular problems which might affect patients’ quality of life.

Omalizumab is a humanized monoclonal antibody which is an FDA-approved treatment of severe allergic asthma and inhibits IgE binding to FcεRI. According to increasing evidence of IgE inhibition, omalizumab was suggested as a therapeutic approach for bullous pemphigoid (BP). Rituximab has been reported to be effective in various autoimmune diseases, including autoimmune bullous dermatoses. A specific protocol for the use of rituximab to treat BP patients is not yet available. There are only small case series and case reports about the efficacy and safety of rituximab in BP. Here we present a young BP patient who responded well to rituximab therapy and was refractory to conventional and omalizumab therapies although he had elevated IgE levels and eosinophilia. Our case supports the knowledge about the effectiveness and safety of rituximab not only in pemphigus but also in BP. On the other hand, although it did not work in our case, omalizumab may be a potentially effective agent in some carefully selected patients with certain subtypes of BP.

Objective: Restless legs syndrome (RLS) is a chronic disorder characterized by an urge to move the legs. In the present study, we investigated the frequency of RLS in patients with psoriasis and tried to obtain the effects of RLS on sleep quality and psychiatric wellbeing. Methods: A total of 114 consecutive psoriasis patients (60 women and 54 men) and 67 control subjects who attended the Department of Dermatology and Venereology were enrolled in the study. A diagnosis of RLS was made according to the criteria of the International RLS Study Group (IRLSSG), and severity was assessed using the IRLSSG severity scale. Sixty-seven of 114 patients answered the Turkish version of Beck Depression Inventory (BDI) and Pittsburgh Sleep Quality Index. Results: We found a higher frequency of RLS in psoriasis patients (11.4%) compared to healthy controls (6.0%), but this prevalence has not reached a statistical significance (p=0.23). Body mass index, BDI score, and bad sleep quality were significantly higher in psoriasis patients compared to healthy controls. Conclusion: Since presence of RLS worsens psoriasis patients’ already bad quality of life, we suggest screening of patients for the presence of RLS, and directing them to a rheumatologist can improve their quality of life.

Behçet's disease (BD) is a chronic, relapsing, inflammatory multisystem disease of unknown etiology. Oral ulcers, genital ulcers, cutaneous lesions, and ocular and articular involvement are the most frequent features of the disease. Mucocutaneous lesions are considered hallmarks of the disease, and often precede other manifestations. Therefore, their recognition may permit earlier diagnosis and treatment with beneficial results for prognosis. BD is particularly prevalent in "Silk Route" populations but has a global distribution. The disease usually starts around the third or fourth decade of life. Sex distribution is roughly equal. The diagnosis is based on clinical criteria, as there is no pathognomonic test. Genetic factors have been investigated extensively, and association with human leukocyte antigen (HLA)-B51 is still known as the strongest genetic susceptibility factor. The T-helper 17 and interleukin (IL)-17 pathways are active, and play an important role, particularly in acute attacks of BD. Neutrophil activity is increased in BD, and the affected organs show a significant neutrophil and lymphocyte infiltration. HLA-B51 association and increased IL-17 response are thought to play a role in neutrophil activation. Treatment is mainly based on the suppression of inflammatory attacks of the disease using immunomodulatory and immunosuppressive agents. Although treatment has become much more effective in recent years with the introduction of newer drugs, BD is still associated with considerable morbidity and increased mortality. Male sex, younger age of onset and increased number of organs involved at the diagnosis are associated with a more severe disease and, therefore, require more aggressive treatment.

Autoimmune bullous diseases are rare, organ-specific, a group of blistering disease of skin and mucous membranes. Recent studies suggest that the frequency of the autoimmune bullous diseases has been increasing. Pemphigus vulgaris and bullous pemphigoid are the most frequently reported autoimmune bullous diseases. High incidence of autoimmune bullous diseases in some ethnic groups such as pemphigus in Ashkenazi Jewish, or in some regions such as pemphigus foliaceus in Brazil has been shown to be related to genetic and environmental factors, respectively. Pemphigus has been reported more frequently in the female gender. Although it is most frequently diagnosed between the ages 50 and 60 in European countries, in the remaining countries in the world, it is seen between the ages of 30 and 50. Bullous pemphigoid is generally seen above 70 years of age. Although overall incidence is slightly higher in females, after the age of 80 years it is more frequent in males. Both pemphigus vulgaris and bullous pemphigoid has a chronic course with recurrences. Mortality risk of the patients with bullous pemphigoid was found at least 2 times higher and the mortality risk of the patients with pemphigus was found approximately 3 times higher than that of the general population. In this review, the results obtained from the epidemiological studies were analyzed according to geographic regions, and especially epidemiologic features of two prevalent autoimmune bullous diseases, pemphigus and bullous pemphigoid have been discussed.

Objective To prepare a dermatologic addendum to the 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides (CHCC2012) to address vasculitides affecting the skin (D-CHCC). The goal was to standardize the names and definitions for cutaneous vasculitis. Methods A nominal group technique with a facilitator was used to reach consensus on the D-CHCC nomenclature, using multiple face-to-face meetings, e-mail discussions, and teleconferences. Results Standardized names, definitions, and descriptions were adopted for cutaneous components of systemic vasculitides (e.g., cutaneous IgA vasculitis as a component of systemic IgA vasculitis), skin-limited variants of systemic vasculitides (e.g., skin-limited IgA vasculitis, drug-induced skin-limited antineutrophil cytoplasmic antibody–associated vasculitis), and cutaneous single-organ vasculitides that have no systemic counterparts (e.g., nodular vasculitis). Cutaneous vasculitides that were not included in the CHCC2012 nomenclature were introduced. Conclusion Standardized names and definitions are a prerequisite for developing validated classification and diagnostic criteria for cutaneous vasculitis. Accurate identification of specifically defined variants of systemic and skin-limited vasculitides requires knowledgeable integration of data from clinical, laboratory, and pathologic studies. This proposed nomenclature of vasculitides affecting the skin, the D-CHCC, provides a standard framework both for clinicians and for investigators.

WOS: 000408928500016, PubMed: 28407292, Internalized stigma is the adoption of negative attitudes and stereotypes of the society regarding a person's illness. It causes decreased self-esteem and life-satisfaction, increased depression and suicidality, and difficulty in coping with the illness. The primary aim of this study was to investigate the internalized stigma state of psoriatic patients and to identify the factors influencing internalized stigma. The secondary aim was to identify the correlation of internalized stigma with quality of life and perceived health status. This multicentre, cross-sectional study comprised 1485 patients. There was a significant positive correlation between mean values of Psoriasis Internalized Stigma Scale (PISS) and Psoriasis Area and Severity Index, Body Surface Area, Dermatological Life Quality Index and General Health Questionnaire-12 (P < 0.001 in all). Lower percieved health score (P = 0.001), early onset psoriasis (P = 0.016), family history of psoriasis (P = 0.0034), being illiterate (P < 0.001) and lower income level (P < 0.001) were determinants of high PISS scores. Mean PISS values were higher in erythrodermic and generalized pustular psoriasis. Involvement of scalp, face, hand, genitalia and finger nails as well as arthropathic and inverse psoriasis were also related to significantly higher PISS scores (P = 0.001). Our findings imply that psoriatic patients experience high levels of internalized stigma which are associated with psoriasis severity, involvement of visible body parts, genital area, folds or joints, poorer quality of life, negative perceptions of general health and psychological illnesses. Therefore, internalized stigma may be one of the major factors responsible from psychosocial burden of the disease.

The data on long-term efficacy, safety and drug survival rates of conventional systemic therapeutics in pediatric psoriasis is lacking. The primary aim of this study is to investigate acitretin, methotrexate, cyclosporin efficacy, safety and drug survival rates in pediatric patients as well as predictors of drug survival. This is a multicenter study including 289 pediatric cases being treated with acitretin, methotrexate and cyclosporin in four academic referral centers. Efficacy, adverse events, reasons for discontinuation, 1, 2- and 3-year drug survival rates, and determinants of drug survival were analyzed. A 75% reduction of Psoriasis Area and Severity Index score or better response rate was obtained in 47.5%, 34.1% and 40% of the patients who were treated with acitretin, methotrexate and cyclosporin, respectively. One-year drug survival rates for acitretin, methotrexate and cyclosporin were 36.3%, 21.1% and 15.1%, respectively. The most significant determinant of drug survival, which diminished over time, was treatment response whereas arthritis, body mass index and sex had no influence. Although all three medications are effective and relatively safe in children, drug survival rates are low due to safety concerns at this age group. Effective disease control through their rational use can be expected to improve survival rates. Pfizer Novartis Merck & Company - Schering Plough Corporation AbbVie Merck & Company Johnson & Johnson - Johnson & Johnson USA - Janssen Biotech Inc

Background Pemphigus vulgaris (PV) is a severe, chronic, and potentially life-threatening autoimmune blistering disease that affects the skin and mucous membranes. Rituximab is a monoclonal anti-CD20 antibody which has been used increasingly in the therapy of PV. Methods The present study sought to test the efficacy and safety of rituximab as an adjuvant therapy by retrospective analysis of clinical and immunological data for 29 patients with PV who were treated with rituximab between 2010 and 2015. Response to therapy, duration of clinical remission, serology of the response, and adverse effects of rituximab were evaluated. Results The mean ± standard deviation (SD) follow-up time was 17.48 ± 13.18 months. In all patients, findings showed either a decrease in antibody titers or that antibodies were completely undetectable after therapy. Rituximab use resulted in a significant reduction in steroid dosage during follow-up. At the end of the follow-up period, 26 patients (96.2%) had achieved complete remission with or without therapy (one patient had no follow-up and one patient had died, most probably as the result of a thromboembolic event). In 44.4% of patients, a clinical relapse occurred after a mean ± SD period of 13.1 ± 4.7 months after the initiation of rituximab therapy. Relapses were managed with additional infusions of rituximab. Conclusions Rituximab is a beneficial and relatively safe adjuvant treatment for PV that facilitates prolonged clinical remission and has a significant steroid-sparing effect.

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. Schering Plough Corporation AbbVie Merck & Company Johnson & Johnson Johnson & Johnson USA Janssen Biotech Inc Merck & Company

Background: Congenital melanocytic naevi (CMN) are present at birth or they develop during the first year of life. In the literature, there is no study prospectively determining the prevalence and dermoscopic patterns of CMN among 0-12 months old infants. There is no data about its frequency in Turkish population. Dermoscopic diagnostic criteria for CMN are not clear. Aim: The aim of this study is to determine the frequency of CMN in our region and to define their dermoscopic properties. Methods: 4289 consecutive infants aged between 0 and 12 months, admitted to pediatric outpatient clinics of Akdeniz University Hospital were included in this study. Infants were first examined by a pediatrician and those with naevi were consulted to a dermatologist. Lesions of CMN were evaluated with camerated dermatoscopy device and images were recorded to a computer. Results: CMN were detected in 0,5% (n:20) of cases. Lower extremity was the most common location (60%), followed by scalp (15%), trunk (10%), upper extremity (10%) and more than one location (5%). Naevi diameter was 1.5-20 cm in 11 cases, smaller than 1,5cm in seven cases and greater than 20cm in two cases. In dermoscopic examination dot structure (n:18) and globular pattern (n:15) were mostly observed. Other findings included multifocal hypopigmentation pattern (n:6), reticular pattern (n:4), homogeneous pattern (n:4), cobblestone pattern (n:2), and parallel furrow pattern (n:1). Conclusion: This is the first prospective study to determine the prevalence and dermoscopic patterns of CMN among 0-12 months old infants admitted to a pediatric outpatient clinic. The frequency of CMN in our region is similar with some of the previous studies. Our Dermoscopic results support that CMN appearing in younger ages is more often characterized by a globular pattern and dot structure.

Behçet's disease (BD) is a chronic, relapsing, and debilitating systemic vasculitis of unknown aetiology with the clinical features of mucocutaneous lesions, ocular, vascular, articular, neurologic, gastrointestinal, urogenital, and pulmonary involvement. The disease is much more frequent along the ancient “Silk Route” extending from Eastern Asia to the Mediterranean basin, compared with Western countries. The disease usually starts around the third or fourth decade of life. Male sex and a younger age of onset are associated with more severe disease. Although the treatment has become much more effective in recent years, BD is still associated with severe morbidity and considerable mortality. The main aim of the treatment should be the prevention of irreversible organ damage. Therefore, close monitoring, early, and appropriate treatment is mandatory to reduce morbidity and mortality. The treatment is mainly based on the suppression of inflammatory attacks of the disease using immunomodulatory and immunosuppressive agents. In this paper, current state of knowledge regarding the therapeutic approaches is outlined. To provide a rational framework for selecting the appropriate therapy along the various treatment choices, a stepwise, symptom-based, evidence-based algorithmic approach was developed.

Serum IL-17 levels and IL-17 mRNA expression have been reported to be higher in psoriatic skin than normal skin. There are very limited data in the literature about difference in the levels of this cytokine in various clinical disease subtypes. We aimed to evaluate whether there is a difference in the level of this cytokine according to clinical subtypes of psoriasis. 70 psoriasis patients (30 plaque psoriasis, 20 guttate psoriasis, and 20 pustular psoriasis) and 50 age- and sex-matched healthy volunteers were included in the study. Serum IL-17 levels were determined by ELISA. Skin biopsies obtained from lesions and non-lesional skin area of 12 patients and healthy individuals (n = 5) were analyzed by quantitative PCR (qPCR) to measure the mRNA levels of IL-17. Statistically, the serum IL-17 levels did not exhibit any difference between the patients and control groups. However, analysis of each subgroup revealed that the IL-17 levels were significantly higher in pustular psoriasis group (10.09 ± 12.6 pg/ml) than controls (4.4 ± 4.1 pg/ml) (p = 0.02). In addition, the IL-17 levels of plaque psoriasis patients with PASI score ≥10 (11.30 ± 6.0 pg/ml) were significantly higher than that of patients with PASI score

We have very limited knowledge about aotuimmune bullous disesases which are important causes of morbidity and mortality. They are generally rare disases in population. The yearly over all incidences of pemphigus and bullous pemhigoid are between 0.5 to 16.1/million and 2.5 to 42.8/million, respectively. Pemphigus vulgaris is the major type of pemphigus and it is most prevalent between ages of 40 and 50. Bullous pemphigoid is tpypically most prevalent in ages of over 70. In this review the results obtained from the studies which are especially about epidemiology of pemphigus and bullous pemphigoid were analised according to geographic regions.

Background Unilateral nevoid telangiectasia (UNT) is a unique vascular dermatosis of ambiguous aetiology. Objective To investigate the role of neurological disorder in pathogenesis of the UNT. Methods We investigated eight consecutive patients with unilateral nevoid telangiectasia. Detailed dermatological and neurological examinations, and magnetic resonance imaging were performed on each patient. In case of presence of dysesthesia over the skin lesion, electroneuromyography was performed to determine any relationships between lesions and peripheral neuropathy. Results All the patients had hypoesthesia over the skin lesion. The cranial magnetic resonance imaging showed subcortical hamartomatous lesions in one patient and demyelinized plaques on the corpus of the caudate nucleus and the pontin area in another. Electroneuromyography evaluation was nonspecific. Conclusion In our study, neurological disorders were associated with UNT. Thus, it can be speculated that neurological disorders might contribute to the development and/or progression of UNT. Patients with UNT should be encouraged for neurological investigation.

Psoriasis is a skin disorder that is associated with arthritis. Sacroiliac joint involvement is considered to be less frequent than the other types of psoriatic arthritis. Additionally, the psoriatic sacroiliitis is considered to be asymmetric in general. We aimed to define the frequency and type of sacroiliac involvement in patients with psoriasis. Patients with psoriasis were included the study. Characteristics of skin, nail and articular involvement were noted. Psoriasis area and severity index was calculated. Antero-posterior pelvic X-rays were obtained and graded by two rheumatologists and a radiologist independently. One hundred and thirty-three patients were included. Thirty-seven of patients (27%) have articular involvement symptomatically. The sacroiliac joint involvement was observed in 34 (26%) of patients. More than one-half of sacroiliac involvement was bilateral while less than one-half was in symptomatic patients regarding sacroiliitis. Fifty-seven percentages of all patients have psoriatic nail involvement. Sacroiliac joint involvement did not show any significant association with psoriatic nail involvement or the severity of skin disease. We found higher frequency of sacroiliac joint involvement and bilateral sacroiliitis in patients with psoriasis. This is in contrast to present information about the association of psoriasis and sacroiliitis. These findings need confirmation by further studies and with more sophisticated techniques such as magnetic resonance imaging.

Objective: The aim of this study was to evaluate the periodontal status of pemphigus vulgaris (PV) patients and compare it with that of healthy controls. We also analysed the association between the periodontal condition and the clinical severity of the disease in PV patients. Study design: Twenty patients (nine women, 11 men; mean ± s.d. age, 42.9±9.8 years) with PV and 22 healthy subjects (eight women, 14 men; mean ± s.d. age, 40.5 ± 12.1 years) were included in the study. The periodontal status of all subjects was evaluated according to the Community Periodontal Index of Treatment Needs (CPITN). PV patients were also assessed for Clinical Severity Score (CSS). Results: The mean CPITN values were observed to be higher in PV patients (2.8 ± 0.7) compared with those of healthy controls (1.0 ± 0.8) (P

Background: Behcet's disease (BD) is a chronic, relapsing, systemic vasculitis of unknown aetiology. The involvement of oral mucosal surfaces represents the onset feature of the disease in the majority of patients. Objective: The aim of this study was to evaluate the periodontal status of BD patients and then compare with recurrent aphthous stomatitis (RAS) patients and healthy controls. We also determined the relationship between the periodontal condition and the clinical severity of the disease in BD patients. Methods: Eighty-six patients with BD, 63 patients with RAS and 82 healthy subjects were included in the study. The periodontal status of all subjects was evaluated according to the community periodontal index of treatment needs (CPITN). BD patients were also assessed for clinical severity score (CSS) as described previously. Results: The mean CPITN were observed to be higher in BD patients (1.79 ± 0.96) compared with RAS patients (1.22 ± 0.87) and healthy controls (1.18 ± 0.98) (p

Summary and conclusions The published evidence on the use of anti-TNF agents in BDconsists mainly of reports of the open use of infliximab,principally evaluated as an add-on therapy. The majority ofpatients suffered from relapsing, posterior segment ocularinflammation, inadequately controlled with available immuno-suppressive therapy. With infliximab, a fast-onset therapeuticeffect was repeatedly observed in patients with sight-threateninginflammation, including patients with retinal vasculitis. Thepaucity of effective and fast-acting therapies particularly forpatients with eye disease underscores the importance of thesefindings. As suggested by three independent, open-label, prospec-tive, self-controlled studies, repetitive infliximab infusions werealso effective in preventing ocular relapses, maintaining visualacuity and tapering immunosuppressive therapy in the majority ofpatients who had an inadequate response or were intolerant toconventional therapy. Infliximab was effective for extra-ocularmanifestations in these patients, as well as in other patients withrecalcitrant orogenital ulcers, arthritis, intestinal or centralnervous system involvement and in a single patient withpulmonary artery aneurysm. With short-term use, there were noserious side effects reported. The only randomized controlled trialwas a 4-week study of etanercept in patients with mucocutaneousmanifestations. Etanercept was beneficial for most of thesemanifestations, but no data are at hand from this study on eyeinvolvement.Anti-TNF agents have a number of disadvantages, includinginduction of potentially serious adverse events and high cost.However, based on the available evidence, physicians may decideto provide anti-TNF therapy to patients with definite BD.However, until results from adequately powered, randomizedcontrolled clinical trials are available, TNF blocking agentsshould be used with caution only for selected patients withsevere disease. Patients with two or more relapses of posterioruveitis per year, low visual acuity due to chronic cystoid macularoedema, or active CNS disease and/or selected patients withintestinal inflammation, or arthritic and mucocutaneous manifes-tations that reduce significantly the quality of life, would fit thiscategory. According to the experience accumulated so far,infliximab seems to be more efficacious than etanercept in diseasemanifestations other than mucocutaneous or joint involvement,while data on adalimumab are very limited. Infliximab oretanercept is recommended as an add-on therapy for selectedpatients with BD, who are refractory or intolerant to traditionalimmunosuppressive regimens. Moreover, a single infusion ofinfliximab (5mg/kg), can be used as a first-line agent for sight-threatening, bilateral posterior eye segment inflammation, whenthe fast-onset of response is considered to be critical to preventfixed retinal lesions and thus permanent visual loss. In those caseswhose ocular relapses are not controlled by azathioprine and/orciclosporin, a maintenance therapy with infliximab at the dose of5mg/kg every 6–8 weeks could be used for up to 2 years, providedno relapses occur between intervals. These recommendations donot constitute treatment guidelines but are intended to improvepractice uniformity and to help physicians in the management ofBD patients, until higher grades of evidence are available.

Objective Cutaneus leishmaniasis, a chronic self-limited disease of the skin, is usually caused by Leishmania Tropica. It is endemic in Southeastern Anatolia. The definitive diagnosis depends on demonstration of the parasites by smear and culture or its identification in tissue section. This study aimed to evaluate clinical and histopathological skin lesions in cutaneous leishmaniasis cases in Antalya, Turkey. Material and method Our study included 28 patients diagnosed with cutaneous leishmaniasis at the Pathology Department of Akdeniz University Medical Faculty. Histopathological sections were stained with Haematoxylin-Eosin, Giemsa or Leishman for visual examination of cellular components by two dermatopathologists. The epidermal (acanthosis, hyper-parakeratosis, atrophy, lymphocytic exocytosis) and dermal changes that may indicate lymphohistiocytic infiltration and granuloma formation were investigated. The parasitic load was classified according to the modified Ridley's parasitic index. Results Out of 28 cases, 11 had hyperparakeratosis, 17 had orthokeratosis, 20 had acanthosis, 4 had epidermal atrophy, and 7 had exocytosis. Typical epithelioid cell granulomas with giant cells and a rim of lymphocytes were present in 16 cases. Leishman-Donovan bodies were extremely rare in typical granulomatous lesions. The other 12 cases showed lymphohistiositic infiltration, giant cells and prominent plasma cells. There were numerous Leishman-Donovan bodies in these lesions. Conclusion We investigated the epidermal and dermal changes that would facilitate the histopathological diagnosis of cutaneous leishmaniasis in this study. We found that atrophy, acanthosis, and orthokeratosis were early stage indicators, while exocytosis, hyperparakeratosis, and atrophy were indicative of late stage disease.

Background Tumor necrosis factor-alpha (TNF-α) antagonist treatment is associated with 1.6 to 27 times higher risk of tuberculosis (TB). Objective To find TB incidence of psoriasis patients treated with TNF- α antagonists and define risk factors related with this condition in a country with moderately high risk of TB. Methods Three hundred seventy psoriasis patients treated by anti-TNF agents in four referral centers were included. The data on the characteristics of the patients, TB history, tuberculosis skin test results, anti-TNF agent type and exposure time, localization of TB, and isoniazide prophylaxis state were analyzed. Results Four patients (1.08%) developed TB, three pulmonary and one gastrointestinal, 2–23 months after initiating anti-TNF agents. Other than the patient with gastrointestinal TB, who was using methotrexate and corticosteroid concomitantly, none had contributing risk factors for TB. Two patients developed pulmonary TB in spite of chemoprophylaxis. Three patients with pulmonary TB completely recovered following antiTB treatment whereas patients with gastroinrestinal TB developed renal failure. Limitations The major limitation of the study is the lack of a diseased control group, which enables us to compare the risk of psoriatics with that of patients having other inflammatory diseases. Conclusion Tuberculosis is a rare but a severe complication of anti-TNF treatment and may develop in spite of chemoprophylaxis. The risk of TB in psoriasis patients in the present study is comparable to literature mostly based on rheumatology patients.

Our goal was to determine, retrospectively, the occurrence of the symptoms of Behcet’s disease in chronological order and the course of the disease. Additionally, probable factors affecting the clinical severity were investigated. A total of 368 patients (171 females and 197 males; aged 41.11 ± 10.9 years) were included in this retrospective cohort study. The chronological order of the clinical manifestations was recorded. Patients were also assessed for clinical severity score. Oral ulcer was the most common manifestation (100 %) followed by genital ulcer (89.4 %), papulopustular lesions (75 %) and articular involvement (60.1 %). Oral ulcer was the most common onset manifestation (66.8 %) followed by genital ulcer (4.9 %), erythema nodosum (3.3 %) and ocular involvement (1.4 %). The duration between the onset symptom and the fulfillment of the diagnostic criteria was 4.67 ± 5.9 years. The duration between the time point of fulfillment of diagnostic criteria and the diagnosis (2.5 ± 2.1 years) was longer in patients having only mucocutaneous lesions (2.8 ± 2.2 years) than in patients having serious organ involvements (1.9 ± 1.6 years; p

Background Recurrent and painful ulcers of the oral mucosa and genital skin/mucosa are the most commonly observed manifestations in patients with Behcet's disease (BD). They affect patients’ quality of life. Because of the effectiveness of granulocyte colony-stimulating factor (G-CSF) in wound healing, it may also be useful for the treatment of oral ulcers (OU) and genital ulcers (GU) of BD. Objective We aimed to determine the efficacy of topically applied G-CSF in the treatment of OU and GU of BD. Methods Seven patients with BD diagnosed according to the criteria of the International Study Group for Behcet's Disease were involved in the study. The patients were observed for 3 months before the study, and all occurrences were recorded during this period. Patients were given topical G-CSF for OU (4 × 120 µg/day, for 5 days) and/or GU (4 × 30 µg/day, for 5 days) and followed-up for 3 months after treatment. No concurrent disease-specific or immunosuppressive topical or systemic drugs were given during the study period. Results G-CSF treatment decreased the healing time and pain of OU and GU in six of seven patients compared with the pretreatment period. However, the effectiveness of the G-CSF treatment on OU and GU healing time and pain severity did not continue during the post-treatment period. Conclusions G-CSF has beneficial effects on the healing duration and pain severity of OU and GU of patients with BD. However, given the high cost, impractical preparation and inability to cure the disease, G-CSF treatment should be chosen only in selected patients.

Behcet’s disease is a chronic, relapsing, systemic vasculitis of unknown etiology with the clinical features of mucocutaneous lesions and ocular, vascular, articular, gastrointestinal, urogenital, pulmonary and neurologic involvement. The disease is particularly prevalent in ‘Silk Route’ populations but has a global distribution. Behcet’s disease effects primarily young subjects. The diagnosis is based on clinical criteria, as there is no pathognomonic test. Besides considerable morbidity, the disease confers an increased mortality, mainly due to large vessel (especially pulmonary arterial) and neurologic involvement as well as bowel perforation. No standard therapy has yet been established for the treatment of Behcet’s disease, and a wide spectrum of therapeutic agents have been used with varying success. Treatment of the disease has become much more effective in recent years with the introduction of newer drugs. This paper will aim to provide an overview of the therapeutic approaches including local and systemic agents for the treatment of Behcet’s disease.

Kaposi's varicelliform eruption is characterized by disseminated vesiculopustules and erosions caused by a herpes virus infection superimposed on a pre-existing dermatosis. The eruption usually occurs in individuals with atopic dermatitis or other pre-existing dermatosis such as Darier's disease, pemphigus foliaceus, mycosis fungoides, Sezary syndrome, benign familial pemphigus, ichthyosis vulgaris, second-degree burns, multiple myeloma, and Grover's disease. We report here a new case of Kaposi's varicelliform eruption in a 38-year-old woman with rosacea. To our knowledge, this is the first case of Kaposi's varicelliform eruption associated with rosacea to be reported.

Papuloerythroderma of Ofuji (PEO) is a disease of elderly men, characterized by intensely pruritic and widespread, red, flat-topped papules with sparing of the body folds and creases (the so-called 'deck-chair' sign). The etiopathogenesis of the disease remains unknown. Psoralen plus UVA (PUVA), topical and systemic corticosteroids, etretinate, cyclosporin and interferon are the main approaches in the treatment of this rare disease. A case of PEO in a 60-year-old man who responded to retinoid plus PUVA (Re-PUVA) treatment is reported here and a review of the therapy with other relevant cases is presented.