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

  • Suzan Demir Pektas Mugla sitki kocman university faculty of medicine
  • Emine Tugba Alatas
  • Gokhan Pektas
  • Volkan Karakus
  • Fatih Mehmet Azik
  • Gursoy Dogan
Keywords: Beta thalassemia major, dermatological disease, hair, skin, nail

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.

References

Modell B, Darlison M. Global epidemiology of haemoglobin disorders and derived service indicators. Bull World Health Organ. 2008;86:480-7.

Skandalis K, Vlachos C, Pliakou X, Gaitanis G, Kapsali E, Bassukas ID. Higher Serum Ferritin Levels Correlate with an Increased Risk of Cutaneous Morbidity in Adult Patients with β-Thalassemia: A Single-Center Retrospective Study. Acta Haematol. 2016;135:124-30.

John B. Porter. Optimizing iron chelation strategies in b-thalassaemia major. Blood Reviews 23 Suppl. 2009;1:3-7.

Dogramaci AC, Savas N, Ozer B, Duran N. Skin diseases in patients with b-thalassemia major. Int J Dermatol. 2009;48:1057-61.

Fahmey SS, Taha G, El-Refaey A, Adly S. Skin Disorders in Egyptian Children with β-Thalassemia Major. J Trop Pediatr. 2017 May 16.

Bomford AB, Munro HN. Ferritin gene expression in health and malignancy. Pathobiology. 1992;60:10-8.

Baldi A, Lombardi D, Russo P, Palescandolo E, De Luca A, Santini D, et al. Ferritin Contributes to Melanoma Progression by Modulating Cell Growth and Sensitivity to Oxidative Stress. Clin Cancer Res. 2005;11:3175-83.

Mansur AT, Aydıngoz IE, Goktay F, Atalay S. Serum Iron and Ferritin Levels in Patients with Vitiligo. Turk derm. 2010;44:153-5.

Iuga AO, Qureshi AA, Lerner EA. Nitric oxide is toxic to melanocytes in vitro. Pigment Cell Res. 2004;17:302-6.

Pourzand C, Watkin RD, Brown JE, Tyrrell RM. Ultraviolet A radiation induces immediate release of iron in human primary skin fibroblasts: the role of ferritin. Proc Natl Acad Sci. 1999;96:6751-6.

Tisma VS, Basta-Juzbasic A, Jaganjac M, Brcic L, Dobric I, Lipozencic J, et al. Oxidative stres and ferritin expression in the skin of patients with rosacea. J Am Acad Dermatol. 2009;60:270-6.

Voskou S, Aslan M, Fanis P, Phylactides M, Kleanthous M. Oxidative stres in β-thalassaemia and sickle cell disease. Redox Biology. 2015;6:226-39.

Farage MA, Miller KW, Berardesca E, Maibach HI. Clinical implications of aging skin: cutaneous disorders in the elderly. Am J Clin Dermatol. 2009;10:73-86.

Denat L, Kadekaro AL, Marrot L, Leachman SA, Abdel-Malek ZA. Melanocytes as Instigators and Victims of Oxidative Stress. J Invest Dermatol. 2014;134:1512-8.

Burgdorf W.H.C, Plewig G, Wolff H.H. Landthaler M. Braun-Falco´s. Dermatology 3rd ed, 2009. SpringerVerlag; 1403-13.

Dalgard F, Svensson A, Holm JØ, et al. Self-reported skin morbidity in Oslo. Associations with sociodemographic factors among adults in a cross-sectional study. Br J Dermatol. 2004;151:452-7.

Lotti T, Ghersetich I, Comacchi C. Cutaneous small-vessel vasculitis. J Am Acad Dermatol. 1998;39:667-87.

Gulen F, Demir E, Tanac R. Successful desensitization of a case with desferrioxamine hypersensitivity. Minerva Pediatr. 2006;58:571-4.

Farage MA, Miller KW, Berardesca E, Maibach HI. Clinical implications of aging skin: cutaneous disorders in the elderly. Am J Clin Dermatol. 2009;10:73-86.

Wollina U, Nowak A. Dermatology in the intensıve care unit. Our Dermatol Online. 2012;3:298-303.

Emre S, Emre C, Akoglu G, Demirseren DD, Metin A. Evaluation of dermatological consultations of patients treated in intensive care unit. Dermatology. 2013;226:75-80.

Fulop T, Tessier D, Carpentier A. The metabolic syndrome. Pathol Biol. 54:375-86.

Kunutsor SK, Apekey TA, Walley J. Ferritin levels and risk of type 2 diabetes mellitus: an updated systematic review and meta-analysis of prospective evidence. Diabetes Metab Res Rev. 2013;29:308-18.

Shalitin S, Carmi D, Weintrob N. Serum ferritin level as a predictor of impaired growth and puberty in thalassemia major patients. Eur J Haematol. 2005;74:93-100.

Published
2018-03-15
Section
Original Research