Abstract

Objective: β-thalassemia major (BTM) is a genetic disorder necessitating frequent blood transfusions, which is characterized by functional and physiological disorders in multiple organs. We aimed to research the prevalence of associated skin, hair, and nail disorders, and their relationship with clinical and laboratory features of the disease in adult BTM patients and healthy control groups. Materials and Methods: We prospectively analyzed the prevalence of associated skin, hair, and nail disorders. We recorded demographic features, laboratory findings skin, hair, and nail disorders in the participants. Results: Atotal of 71 BTM patients (36 males and 35 females) and 50 age and sex matched healthy individuals (36 females and 36 males) were included in the study. 32 (45.1%) of the patients and 3 (4.2%) of the control groups had hair, 26 (36.6%) of the patients and 6 (8.3%) of control groups had nail diseases. The most common skin findings xerosis were determined in 66 (93%), hair findings, telogen effluvium were determined in 16 (22.5%), and nail findings, dystrophic nail were determined 9 (12.7%) in patients. BTM patients had significantly higher fruquent xerosis, scar, pigmentation disorders, nevus, pruritus, infections, nail and hair disorders than control groups. Average age of patients with pruritus and nevus were significantly enhanced. Postinflammatory hyperpigmentation and melanocytic nevus in male patients had more higher than female patients. Telogen effluvium in female patients was more higher than male patients. Ferritin levels of the patients with ephelide, diffuse bronze pigmentation (DBP) and telogen effluim had significantly enhanced. Conclusion: Age, gender, medication history and ferritin level are important factors for the development of dermatological disorders among BTM patients.

Highlights

  • Amaç: β-talasemi majör (BTM), çoklu organlarda fonksiyonel ve fizyolojik bozukluklar ile karakterize, sık kan transfüzyonu gerektiren genetik bir hastalıktır

  • The aim was to investigate the relationship between the frequency of skin, hair and nail disorders associated with beta thalassemia major (BTM) patients and, clinical and laboratory characteristics of dermatological disorders

  • According to the other two studies, we found a higher incidence of xerosis in our study; we believe that only adult BTM patients are involved in the study, and the skin structure is deteriorated due to the high average age and the long disease duration, and higher rate of coexistence of comorbid conditions in the study group

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Summary

Introduction

Amaç: β-talasemi majör (BTM), çoklu organlarda fonksiyonel ve fizyolojik bozukluklar ile karakterize, sık kan transfüzyonu gerektiren genetik bir hastalıktır. The aim was to investigate the relationship between the frequency of skin, hair and nail disorders associated with BTM patients and, clinical and laboratory characteristics of dermatological disorders. This prospective, randomized study has been approved by the ethics committee of Mugla Sitki Kocman University. Patients' age, gender, skin, hair and nail diseases, family history, thalassemia period, comorbid diseases, medications used and laboratory examination results (complete blood count, biochemical values and ferritin level) were recorded. A p-value less than 0.05 was considered to be statistically significant

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