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BACKGROUND: Previous reports indicate that juvenile Behçet's disease (BD) may have a different course than adult BD. However, as a direct comparison with adult Behçet patients has only been made in a limited number of studies, the issue is still controversial. OBJECTIVES: The primary aim of our study was to compare clinical manifestations in a large cohort of juvenile and adult Behçet patients registered in a single centre. The secondary aim of our study was to compare the data of newly diagnosed patients registered between 1998 and 2020 with the data of those registered between 1976 and 1997. METHODS: Data were collected retrospectively from medical records of patients registered between 1998 and 2020. Juvenile BD was defined as fulfilment of International Criteria for Behçet's Disease at or before 16 years of age. RESULTS: A similar course of disease was noted in juvenile and adult Behçet patients with no significant difference in the frequency of mucocutaneous findings, major organ involvement, and positivity of the pathergy test. A comparison of the periods, 1976-1997 and 1998-2020, revealed no significant difference in the prevalence of mucocutaneous lesions and major organ involvement. CONCLUSIONS: Our results indicate that juvenile and adult Behçet patients have a similar course with a similar frequency of clinical manifestations. Contrary to reports suggesting an overall tendency to milder disease over time, no decrease in the risk of major organ involvements was observed. A significant trend towards a decline in pathergy test positivity was noted.
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Síndrome de Behçet , Humanos , Adulto , Síndrome de Behçet/diagnóstico , Síndrome de Behçet/epidemiologia , Síndrome de Behçet/complicações , Estudos Retrospectivos , Seguimentos , PrevalênciaRESUMO
BACKGROUND: Prevalences of sensitivity to contact allergens change over time as a result of variations in allergen exposure. OBJECTIVES: The aim of the study was to describe trends in sensitisation to allergens of the European baseline series in Turkey between 2013 and 2019. METHODS: The patch test results of 1309 patients with suspected allergic contact dermatitis (ACD) were analysed retrospectively, and the prevalence of contact allergies to European baseline series allergens was compared with previous data (for 1998-2005). RESULTS: A total of 534 (40.8%) patients exhibited one or more positive patch test reaction. The five most frequent allergens were nickel sulfate (19.6%), potassium dichromate (6.5%), cobalt chloride (6%), Myroxylon pereirae resin (5%), and p-phenylenediamine (PPD; 3.7%). Statistically significant increases in the prevalence of sensitisation to methylchloroisothiazolinone/methylisothiazolinone (MCI/MI; P < .001), Myroxylon pereirae resin (P < .001), PPD (P = .008), and fragrance mix I (P = .036) were observed in the 2013 to 2019 period compared with the 1998 to 2005 period. Conversely, positive reactions to neomycin sulfate (P = .029), clioquinol (P = .031), and primin (P = .001) decreased significantly. CONCLUSION: This study provides a comprehensive profile of ACD in Turkey. The remarkable increase in the MI and MCI/MI contact allergy observed underlines the urgent need for regulatory measures to reduce exposure to MI and MCI/MI in Turkey.
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Dermatite Alérgica de Contato/diagnóstico , Dermatite Alérgica de Contato/epidemiologia , Testes do Emplastro , Adolescente , Adulto , Distribuição por Idade , Idoso , Idoso de 80 Anos ou mais , Alérgenos/efeitos adversos , Criança , Pré-Escolar , Dermatite Alérgica de Contato/etiologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Conservantes Farmacêuticos/efeitos adversos , Prevalência , Estudos Retrospectivos , Distribuição por Sexo , Turquia/epidemiologia , Adulto JovemRESUMO
BACKGROUND: The differentiation between the pemphigoid diseases is essential for treatment and prognosis. In Turkey, data on the incidence of these diseases are insufficient. Our aim in this study is to determine the incidence, demographics and clinical characteristics associated with diseases of the pemphigoid group. METHODS: We prospectively analysed 295 patients with pemphigoid who visited dermatology clinics of tertiary referral hospitals in 12 different regions of Turkey within a year. The diagnosis was based on clinical, histopathological, direct immunofluorescence (DIF) and serological (multivariant enzyme-linked immunosorbent assay [ELISA], indirect immunofluorescence and mosaic-based BIOCHIP) examinations. Clinical and demographic findings, aetiological factors and concomitant diseases observed in the patients were recorded. RESULTS: A total of 295 (female/male ratio: 1.7/1) patients with pemphigoid were diagnosed in 1-year period. The overall incidence rate of pemphigoid diseases was found to be 3.55 cases per million-years. The ratio of pemphigoid group diseases to pemphigus group diseases was 1.6. The most common pemphigoid type was bullous pemphigoid (BP, 93.2%). The others were epidermolysis bullosa acquisita (3.1%), pemphigoid gestationis (2.4%), linear IgA disease (1%) and mucous membrane pemphigoid (0.3%). The most common (26.8%) possible trigger of the bullous pemphigoid was gliptin derivative drugs. The most common concomitant diseases with pemphigoid were cardiovascular (27.8%) and neurological diseases (23.7%). CONCLUSIONS: This study showed that the increased frequency of bullous pemphigoid reversed the pemphigoid/pemphigus ratio in Turkey. Further studies are warranted regarding the reasons for this increase.
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Penfigoide Bolhoso/diagnóstico , Penfigoide Bolhoso/epidemiologia , Pênfigo/diagnóstico , Pênfigo/epidemiologia , Adolescente , Adulto , Distribuição por Idade , Idoso , Idoso de 80 Anos ou mais , Criança , Pré-Escolar , Feminino , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Distribuição por Sexo , Turquia/epidemiologia , Adulto JovemRESUMO
Sterol-C4-methyl oxidase (SC4MOL) deficiency was recently described as an autosomal recessive cholesterol biosynthesis disorder caused by mutations in the MSMO1 (sometimes also referred to as SC4MOL) gene. To date, 5 patients from 4 unrelated families with SC4MOL deficiency have been reported. Diagnosis can be challenging as the biochemical accumulation of methylsterols can affect global development and cause skin and ocular pathology. Herein, we describe 2 siblings from a consanguineous Turkish family with SC4MOL deficiency presenting with psoriasiform dermatitis, ocular abnormalities (nystagmus, optic hypoplasia, myopia, and strabismus), severe intellectual disability, and growth and motor delay. We undertook whole-exome sequencing and identified a new homozygous missense mutation c.81A>C; p.Asn27Thr in MSMO1. Segregation analysis in all available family members confirmed recessive inheritance of the mutation. The siblings were treated with a combination of oral and topical statin and cholesterol which resulted in clinical improvement. This study demonstrates how genomics-based diagnosis and therapy can be helpful in clinical practice.
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Dermatite/genética , Oxigenases de Função Mista/genética , Mutação de Sentido Incorreto , Psoríase/genética , Criança , Colesterol/biossíntese , Colesterol/sangue , Colesterol/deficiência , Colesterol/uso terapêutico , Consanguinidade , Oftalmopatias/genética , Feminino , Genes Recessivos , Transtornos do Crescimento/genética , Homozigoto , Humanos , Inibidores de Hidroximetilglutaril-CoA Redutases/uso terapêutico , Deficiência Intelectual/genética , Masculino , Oxigenases de Função Mista/deficiência , Linhagem , Rosuvastatina Cálcica/uso terapêutico , Irmãos , Triglicerídeos/sangue , Turquia , Sequenciamento do ExomaRESUMO
Folliculotropic mycosis fungoides (FMF) is characterized by a broad clinical spectrum and worse prognosis compared to classical MF. This study aimed to evaluate the clinical characteristics, treatment modalities and long-term outcome and risk factors for progression and survival of FMF patients. We conducted a single-center retrospective study and reviewed 53 patients diagnosed with FMF between 1990 to 2019 in a referral center at Ankara University, Turkey. Regarding to stage at diagnosis, 24 patients (45.3%) had advanced-stage disease (≥IIB). Follicular papules was observed in 66% and alopecia in 49.1% of the cases. Forty-three patients (81.1%) suffered from pruritus. The majority of the patients (92.4%) had at least one systemic therapy. Complete remission was achieved in 24.5% of the patients. The median time of overall survival (OS) was 50 months (range 9-324 months) and 5-year and 10-year OS was 83% and 69%, respectively. Twenty-eight (52.3%) patients progressed to more advanced stages and seven (13.2%) patients died due to MF during the follow-up period. FMF is associated with a progressive course and in most patients, skin-directed therapies were found to be inefficient to control the disease and multiple systemic therapeutic agents were required to control the disease.
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Micose Fungoide , Neoplasias Cutâneas , Humanos , Micose Fungoide/diagnóstico , Micose Fungoide/terapia , Estudos Retrospectivos , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/terapia , Centros de Atenção Terciária , TurquiaRESUMO
INTRODUCTION: Increasing the numbers of patch testing in suspected children increases the rate of diagnosis of allergic contact dermatitis and the identification of clinically relevant allergens. OBJECTIVE: The aim of this study was to identify the most frequently observed allergens among Turkish children and adolescents patch-tested in 2013-2023. METHODS: The patch test results of 160 patients (age ≤18) were analyzed retrospectively. The frequency of contact allergens and distribution of positive results in terms of sex, age group (children and adolescents), and the presence of atopic dermatitis (AD) were identified. RESULTS: Forty-nine patients (30.6%) (34 girls and 15 boys) exhibited positive patch test reactions to a minimum of one allergen, and contact sensitivity was statistically significantly higher in girls (P=0.034). The five most frequent allergens were nickel sulfate (10.6%), MCI/MI (8.1%), cobalt chloride (5.6%), p-phenylenediamine (PPD) (5%), and MI (3.5%). No significant association was observed between patch test positivity and age groups (P>.05). Nickel sulfate sensitivity was significantly higher in girls than in boys (P=.043). A positive reaction was detected in 31.3% of patients with AD and in 33.7% of those without (P>.05), and a statistically significant relationship was observed between contact sensitivity to fragrance allergens and AD (P=.046). CONCLUSION: Metals and preservatives represent the most frequent allergens in Turkish children and adolescents. Metal sensitivity is expected to decrease as legislation is enforced. Regulatory measures are now required to reduce MI and MCI/MI contact allergy in Turkey.
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INTRODUCTION: The COVID-19 pandemic drastically changed the priorities in healthcare services; outpatient management of acne has changed during this period. OBJECTIVES: We aimed to investigate treatment practices, outcomes and identify modified follow-up schedules applied during the pandemic. METHODS: The patients who were admitted to dermatology outpatient clinic between March 13 and July 13, 2020, were included. Patients who were admitted between March 13 and July 13, 2019, were served as controls for the study. For each patient, age, gender, treatment protocols, treatment intervals, compliance with the treatment, treatment modifications, and adverse events were recorded. RESULTS: The total number of acne patients admitted to dermatology outpatient clinics during the pandemic period was 278 and consisted of 12.3% (278) of all admissions. Isotretinoin treatment was started in only 16 (5.8%) of the patients. The proportion of patients who were under follow-up was significantly higher during the pandemic period (P < 0.005). There was no difference between the pandemic period and the non-pandemic period in terms of starting isotretinoin treatment (P > 0.05). During pandemic period, 79% of the patients who used isotretinoin were followed-up every two or more months. Extended follow-up intervals showed no difference for detecting side effects (P > 0.05). CONCLUSIONS: Acne patients constitute an important part of dermatology outpatient clinics. During the pandemic period, majority of acne patients came for follow-up. Extended follow-up periods were adopted by physicians and were found safe and effective in the current study. Thus, isotretinoin treatment seems efficacious and safe during pandemic period.
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OBJECTIVES: This study aims to compare the sensitivity of the intradermal application of the pathergy test with the "three-step pathergy test". PATIENTS AND METHODS: The study included 60 patients with Behçet's disease (BD) (23 males, 37 females; mean age 33.9±9.7 years; range, 11 to 57 years) and 100 patients with recurrent aphthous stomatitis as the control group (39 males, 61 females; mean age 32.4±12.1 years; range, 11 to 66 years). Simultaneous intradermal pathergy test and three-step pathergy test were applied to each patient. RESULTS: Twenty-six patients (43.3%) with BD had positive three-step pathergy test and 18 (30%) of them had positive intradermal pathergy test. A statistically significant relationship was found between the three-step pathergy test positivity and male sex while no relationship was detected between the disease activity and the positivity of the pathergy test. In the three-step pathergy test, pathergy positivity with intramuscular method was statistically significantly higher than with intravenous method. CONCLUSION: The three-step pathergy test in patients with BD was found to have higher sensitivity compared with the intradermal pathergy test. The intramuscularly applied pathergy test was the main cause of increase in sensitivity. Further studies are needed to develop new applications of pathergy test that would increase the sensitivity and which are also easy to apply.
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BACKGROUND AND OBJECTIVE: Less than 5% of cases of mycosis fungoides (MF) present with a cytotoxic/suppressor CD8+ phenotype. This study aimed to evaluate the clinical characteristics, treatment modalities, and clinical course in CD8+ MF patients. METHODS: In a retrospective analysis of 353 MF patients in a referral center at Ankara University, Turkey, 29 patients that were diagnosed with CD8+ MF were included in the study. RESULTS: CD8+ MF cases constituted 8.2% of all MF patients. The age at the time of diagnosis ranged between 6 and 81 years with a median value of 46 years. The female-to-male ratio was 1.41. Patients presented with erythematous scaly (69%), hyperpigmented (58.6%), poikilodermic (17.2%), and hypopigmented (17.2 %) patches/plaques. The most common sites of involvement were the trunk and lower extremities. The most common comorbidity was hypertension (24.1%, n: 7) with 13 patients (44.8%) having a history of at least one autoimmune disease. At the time of diagnosis, 93.2% of the patients had early-stage disease, and 6.8% of the patients had advanced stage. The mean follow-up period was 6.68 ± 6.04 years (range 1-28 years). Most of the patients were treated with skin-directed therapies. Complete remission was achieved in 17 (58.6%) patients, eight (27.6%) patients had partial remission, and four (13.8%) patients had stable disease. CONCLUSIONS: We concluded that CD8+ MF is associated with an indolent course and in most patients, skin-directed therapies were found to be efficient to control the disease.