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.
Sterol-C4-methyl oxidase (SC4MOL) deficiency was recently described as an autosomal recessive cholesterol biosynthesis disorder caused by mutations in the <i>MSMO1</i> (sometimes also referred to as <i>SC4MOL</i>) 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 <i>MSMO1</i>. 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.
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.
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.
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-tomale 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 earlystage disease, and 6.8% of the patients had advanced stage. The mean follow-up period was 6.68 AE 6.04 years (range 1-28 years). Most of the patients were treated with skindirected 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.
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