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Analysis of Skin, Hair and Nail Diseases in the Adults with Beta Thalassemia Major

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Analysis of Skin, Hair and Nail Diseases in the Adults with Beta Thalassemia Major Original Investigation / Özgün Araştırma 128 Analysis of Skin, Hair and Nail Diseases in the Adults with Beta Thalassemia Major Erişkin Beta Talasemi Major Hastalarında Eşlik Eden Deri, Saç ve Tırnak Bulgularının Araştırılması Suzan Demir Pektas1, Emine Tugba Alatas1, Gokhan Pektas2, Volkan Karakus2, Fatif Mehmet Azik3, Gursoy Dogan1 1 Department of Dermatology, Mugla Sitki Kocman University Faculty of Medicine, Mugla, Turkey 2 Department of Hematology, Mugla Sitki Kocman University Faculty of Medicine, Training and Research Hospital, Mugla, Turkey 3 Department of Pediatric Hematology, Mugla Sitki Kocman University Faculty of Medicine, Mugla, Turkey ABSTRACT ÖZET Objective: β-thalassemia major (BTM) is a genetic disorder necessitating Amaç: β-talasemi majör (BTM), çoklu organlarda fonksiyonel ve fizyolojik frequent blood transfusions, which is characterized by functional and bozukluklar ile karakterize, sık kan transfüzyonu gerektiren genetik bir physiological disorders in multiple organs. We aimed to research the hastalıktır. Yetişkin BTM hastalarında ve sağlıklı bireylerde kontrollerde cilt, prevalence of associated skin, hair, and nail disorders, and their relationship saç ve tırnak hastalıklarının yaygınlıklarını ve bunların klinik ve laboratuvar with clinical and laboratory features of the disease in adult BTM patients and özellikleriyle olan ilişkilerini araştırmayı amaçladık. healthy control groups. Materyal ve Yöntem: İleriye dönük olarak, buna bağlı deri, saç ve tırnak Materials and Methods: We prospectively analyzed the prevalence of hastalıklarının yaygınlığını analiz ettik. Katılımcıların demografik özellikleri, associated skin, hair, and nail disorders. We recorded demographic features, laboratuar bulguları, saç, tırnak ve saç bulgularını kaydettik. laboratory findings skin, hair, and nail disorders in the participants. Bulgular: Araştırmaya toplam 71 BTM hastası (36 erkek ve 35 kadın) ve 50 yaş Results: Atotal of 71 BTM patients (36 males and 35 females) and 50 age and ve cinsiyet ile uygun sağlıklı bireyler (36 kadın ve 36 erkek) dahil edildi. sex matched healthy individuals (36 females and 36 males) were included in Hastaların 32’sinde (%45.1) ve kontrol gruplarının 3’ünde (%4.2) saç bulguları, the study. 32 (45.1%) of the patients and 3 (4.2%) of the control groups had hastaların 26’sında (%36.6) ve kontrol gruplarının 6’sında (%8.3) tırnak hair, 26 (36.6%) of the patients and 6 (8.3%) of control groups had nail bulguları tespit edildi. En yaygın deri bulguları kserozis 66 (% 93), saç bulguları, diseases. The most common skin findings xerosis were determined in 66 telogen effluvium 16 (% 22.5), tırnak bulguları, distrofik tırnak 9 (% 12.7) (93%), hair findings, telogen effluvium were determined in 16 (22.5%), and nail hastada tespit edildi. BTM'li hastalarda, kontrol grubuna göre anlamlı findings, dystrophic nail were determined 9 (12.7%) in patients. BTM patients derecede yüksek sıklıkta kserozis, yara, pigmentasyon bozukluğu, nevüs, had significantly higher fruquent xerosis, scar, pigmentation disorders, nevus, pruritus, enfeksiyon, tırnak ve saç hastalıkları vardı. Pruritus ve nevüs pruritus, infections, nail and hair disorders than control groups. Average age hastalarının ortalama yaş önemli derecede yüksekti. Erkek hastalarda of patients with pruritus and nevus were significantly enhanced. postenflamatuvar hiperpigmentasyon ve melanositik nevüs, kadın hastalardan Postinflammatory hyperpigmentation and melanocytic nevus in male patients daha yüksekti. Kadın hastalarda telogen effluvium, erkek hastalardan daha had more higher than female patients. Telogen effluvium in female patients yüksekti. Efelid, diffuse bronz pigmentasyon (DBP) ve telojen effluim bulunan was more higher than male patients. Ferritin levels of the patients with hastaların ferritin düzeyleri anlamlı olarak yüksekti. ephelide, diffuse bronze pigmentation (DBP) and telogen effluim had Sonuç: BTM'li hastalarda deri hastalıklarının gelişiminde yaş, cinsiyet, ilaç significantly enhanced. öyküsü ve ferritin düzeyi önemli faktörlerdir. Conclusion: Age, gender, medication history and ferritin level are important factors for the development of dermatological disorders among BTM patients. Key Words: Beta thalassemia major, dermatological disease, hair, skin, nail Anahtar Sözcükler: Beta talasemi major, dermatolojik hastalık, saç, cilt, tırnak Received: 02.23.2018 Accepted: 03.01.2018 Geliş Tarihi: 23.02.2018 Kabul Tarihi: 01.03.2018 This article accepted in 2017 EADV congress as an e-poster. Address for Correspondence / Yazışma Adresi: Suzan Demir Pektas, M.D. Department of Dermatology, Mugla Sitki Kocman University Faculty of Medicine 48000, Mugla, Turkey E-mail: [email protected] ©Telif Hakkı 2018 Gazi Üniversitesi Tıp Fakültesi - Makale metnine http://medicaljournal.gazi.edu.tr/ web adresinden ulaşılabilir. ©Copyright 2018 by Gazi University Medical Faculty - Available on-line at web site http://medicaljournal.gazi.edu.tr/ doi:http://dx.doi.org/10.12996/gmj.2018.35 Demir Pektas et al. GMJ 129 2018; 29: 128-133 Dermatological disease in beta thalassemia major INTRODUCTION RESULTS Thalassaemia is the most common monogenic hematologic Of the 71 patients included in the study, 35 were female, 36 were hereditary disease in the World (1). In patients with beta thalassemia male and the average age was 29.6 ± 8.3 years and the 72 healthy major (BTM); without transfusion and chelation treatment, life control included in the study, 36 were female, 36 were male and the expectancy from the time of diagnosis is measured in months. In average age was 29.5±8.9 years (p>0.05). The comparison of patients, iron accumulation develops in the tissues associated with demographic feature and laboratory findings of the participants are continuous blood transfusions, low hepsin levels and ineffective summarized in Table 1. erythropoiesis and consequently oxidative stress occurs (2). With this vicious circle, functional and physiological disorders occur in many Table 1. Clinical and demographic characteristics of organs such as skin, liver, heart, pancreas, gonad, pituitary and thyroid subjects (3, 4). In the literature, there are few studies about the frequency of Patients dermatological diseases in patients with BTM (2, 4, 5). Beside this, Characteristic of partipicants (n:71) Controls (n:72) p progressively when the increase of iron accumulation in the body is Age(years) (mean±SD) 29.6±8.3 29.54±8.965 0.895* considered, as far as we know there is no further research about dermatological disorders in only adult BTM patients. Gender, Male n(%) 36 (50.7) 36 (50) 0.933**** In our research, the aim was to investigate the relationship Family history 47(%66.2) - <0.001*** between the frequency of skin, hair and nail disorders associated with <0.001*** Hematocrit (%) 28.6±3.1 40.2±2.4 BTM patients and, clinical and laboratory characteristics of * <0.001*** dermatological disorders. Hemoglobin (gr/dl) 9.0±0.9 13.4±1.3 * <0.001*** MATERIALS and METHODS Ferritin (ng/mL) 1337.6±1298.5 70.5±15.9 * Comorbid conditions 62 (87.3%) This prospective, randomized study has been approved by the ethics committee of Mugla Sitki Kocman University. This study was Osteoporosis 48(67.6%) practised between the dates May-November 2016, at Mugla Sitki Diabetes Mellitus 18(25.4%) Kocman University Training and Research Hospital Thalassemia Center, cross-sectionally in 71 adult BTM patients under regular follow-up and Hypothyroidism 11(15.5%) treatment and 72 healthy controls. Written consent form was obtained Gastritis 9(12.7%) from all the partipicants included in the study. The data were obtained Depression 8(11.3%) from patient follow-up files and the hospital automation system. Dermatological diseases were practised according to ICD-10 system. All Hyperlipidemia 6(8.5%) partipicants’s skin, hair and nails were examined by a dermatologist. Epilepsy 5(7%) Patients' age, gender, skin, hair and nail diseases, family history, thalassemia period, comorbid diseases, medications used and Hearing loss 5(7%) laboratory examination results (complete blood count, biochemical Neurofibromatosis 5(7%) values and ferritin level) were recorded. Partipicants with a number of melanocytic nevus >20 were included in the study in terms of Mental retardation 4(5.6) melanocytic nevus. Direct microscopic examination and/or culture Asthma 4(5.6%) and/or pathologic examination were performed on the patients who were seen as required, by taking samples from the lesion area Hypogonadism 3(4.2%) (incisional biopsy, scraping and swab material). Chelator agents 70 (98.5%) Statistics Deferasirox 56 (78.9%) To complete statistical assessments, the Statistical Package for Deferiprone 19 (26.8%) Social Sciences (SPSS) software for Windows 22 (IBM SPSS Inc., Chicago, Deferoxamine 16 (22.5%) USA) was used. Quantitative data were shown as mean ± standard Non-chelator agents deviation (SD) and qualitative data were shown as case number and percentile slice. A Pearson chi-squared or Fisher exact test was used to evaluate categorical variables. The distribution of the data was checked Osteoporosis treatment 48(67.6%) by the Kolmogorov Smirnov test. The mean differences of continuous Levothyroxine 10(14.1%) variables with normal distribution between groups were compared with an independent sample student- t-test and for those with non-normal Metformin 8(11.3%) distribution were compared with the Mann–Whitney U test when Insulin 7(9.9%) applicable. A p-value less than 0.05 was considered to be statistically Androgen 6(8.5%)
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