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Cutaneous Manifestation of Β‑Thalassemic Patients Mohamed A [Downloaded free from http://www.mmj.eg.net on Thursday, January 14, 2021, IP: 156.204.184.20] Original article 267 Cutaneous manifestation of β‑thalassemic patients Mohamed A. Gaber, Marwa Galal Departement of Dermatology and Venereology, Objective Menoufia University, Menoufia, Egypt The aim was to study the prevalence of common dermatological problems in patients with Correspondence to Marwa Galal, MBBCh, β‑thalassemia major to help rapid treatment and prevent complications. Shiben Elkom, Menoufia, Background Egypt β‑Thalassemia major affects multiple organs and is associated with considerable morbidity Tel: +20 100 276 8385; Postal code: 32511; and mortality. In β‑thalassemia, a wide spectrum of skin diseases was identified, which were e‑mail: [email protected] caused by both the hemoglobin disorder and the complications of treatment. Patients and methods Received 07 August 2018 Revised 10 September 2018 This cross‑sectional study included 105 Egyptian patients (50 female individuals and 55 male Accepted 23 September 2018 individuals) with transfusion‑dependent β‑thalassemia major in the period spanning from June Published 25 March 2020 2017 to February 2018. The study was performed on child and adult patients of β‑thalassemia Menoufia Medical Journal 2020, 33:267–271 who presented to the hematology clinic, Menoufia University hospital. Skin examination of each patient was carried out, and any skin disease present was recorded. Results The main skin disorders that were noticed in decreasing order of frequency were pruritus (34.4%), xerosis (24.8%), urticaria (21.1%), freckles (17.1%), tinea infections (11.6%), pitriasis alba reported in 10.5%, scars (10.5%), hypersensitivity to deferoxamine pump (9.5%), herpes simplex (9.5%), acne vulgaris (8.6%), miliaria (6.7%), contact dermatitis (4.8%). Conclusion Skin diseases were frequent among patients with β‑thalassemia major. The most common skin lesions in our patients were pruritus, xerosis, and hyperpigmentation. Careful skin examination of thalassemia patients is required to provide early diagnosis of dermatological diseases and a better quality of life. Keywords: β‑Thalassemia, cutaneous manifestations, iron overload, pruritus, xerosis Menoufia Med J 33:267–271 © 2020 Faculty of Medicine, Menoufia University 1110‑2098 β Introduction condition [3,4]. ‑Thalassemia major affects multiple organs and is associated with considerable morbidity β‑Thalassemia is a blood disorder that reduces the and mortality. In β‑thalassemia, a wide spectrum of production of hemoglobin. Hemoglobin is the skin diseases was identified, which were caused by iron‑containing protein in red blood cells that carries both the hemoglobin disorder and the complications oxygen to cells throughout the body. In people with of treatment [5]. A lot of literature has been written on β‑thalassemia, low levels of hemoglobin lead to a lack of various aspects of β‑thalassemia, but little information oxygen in many parts of the body. Affected individuals about the cutaneous manifestation of this disease. The also have a shortage of red blood cells (anemia), which aim of this study was to document the frequency of can cause pale skin, weakness, fatigue, and more serious β β skin diseases among patients with ‑thalassemia major complications [1]. ‑Thalassemia is a fairly common to help rapid treatment and prevent complications. blood disorder worldwide. Thousands of infants with β‑thalassemia are born each year. β‑Thalassemia occurs most frequently in people from Mediterranean countries, North Africa, the Middle East, India, Patients and methods Central Asia, and Southeast Asia [2]. Thalassemia This cross‑sectional study was carried out on patients of is caused by faulty genes that affect the production β‑thalassemia who presented to Menoufia University of hemoglobin. A child can only be born with the hospital during the period spanning from June 2017 condition if they inherit these faulty genes from both to February 2018. The study was conducted on 105 parents. For example, if both parents have the faulty gene that causes β‑thalassemia major, there’s a 25% This is an open access journal, and articles are distributed under the terms chance of each child they have being born with the of the Creative Commons Attribution‑NonCommercial‑ShareAlike 4.0 condition. The parents of a child with the condition License, which allows others to remix, tweak, and build upon the work are usually carriers of thalassemia. This means they non‑commercially, as long as appropriate credit is given and the new only have one of the faulty genes that causes the creations are licensed under the identical terms. 1110-2098 © 2020 Faculty of Medicine, Menoufia University DOI: 10.4103/mmj.mmj_247_18 [Downloaded free from http://www.mmj.eg.net on Thursday, January 14, 2021, IP: 156.204.184.20] 268 Menoufia Medical Journal, Volume 33 | Number 1 | January‑March 2020 Table 1 Demographic characteristics of the studied groups transfused β‑thalassemia major patients. The study was β Sociodemographic data n (%) performed on child and adult patients of ‑thalassemia Sex who attended to Menoufia University hospital. All Male 55 (52.4) patients underwent detailed history evaluation, Female 50 (47.6) including age, sex, and duration of illness. All patients Age (years) were examined to record any dermatological disorders. Mean±SD 18.4±13.3 The patients did not complain about having any Range 2‑50 skin problems, but skin lesions were discovered on examination. Table 2 Duration of thalassemic disease in the patients of the study The work was started after obtaining approval from Duration of the disease the department of dermatology and andrology and Mean±SD 16.5±4.6 Range 1‑59 years the research ethics committee of Menoufia Faculty of Medicine. A self‑report questionnaire was given to participants after obtaining their consent. The xerosis (24.8%), urticaria (21.1%) freckles (17.1%), questionnaire was designed by the investigator. tinea infections (11.6%), pitriasis alba reported Each patient was examined and asked to fill the in 10.5%, scars (10.5%), hypersensitivity to questionnaire to ensure that all gathered information deferoxamine pump (9.5%), herpes simplex (9.5%), was kept confidential and the participant would be kept acne vulgaris (8.6%), miliaria (6.7%), and contact anonymous; each questionnaire was handed over in an dermatitis (4.8%), as shown in Table 3. envelope, and, after filling it, the participant sealed the envelope and put it in a basket containing other sealed envelopes. Discussion All patients in the study had thalassemia for more than Thalassemia is one of the most common single‑gene 1 year. This study included 105 patients aged from 2 disorders worldwide, in which hemoglobin beta chain to 60 years, as thalassemia major, which is the severe production is decreased. Today, the life expectancy of form of thalassemia, becomes apparent before 2 years thalassemia patients is increased because of a variety of age, but thalassemia intemedia and thalassemia of treatment methods; however, treatment‑related minor are less severe and will appear later in life. We complications have also increased. Profound anemia excluded patients who were more than 60 years of age, and severe hemosiderosis cause functional and immune‑compromised patients due to other causes such physiological abnormalities in various organ systems. as diabetes mellitus, hepatitis C virus and AIDS, and This study was conducted to assess the frequency of patients with other associated blood or immunological cutaneous and mucosal manifestations in patients with β disease, to avoid other manifestations caused by aging, ‑thalassemia [6]. lowered immunity and other blood diseases. Iron overload causes significant morbidity and The results were collected and entered into the computer mortality in patients with transfusion‑dependent β using SPSS (statistical package for the social sciences) ‑thalassemia [7]. The higher standards of care in β program for statistical analysis (version 20; Inc., ‑thalassemia have led to a significant increase in Chicago, Illinois, USA). the life expectancy in severely affected patients. Enhanced years of survival have led to the unmasking Descriptive statistics in the form of mean, SD, number of management‑related complications, which were and % were applied. infrequently encountered [8]. Pruritus, which occurred in 34.3% of our patients, was the most common cutaneous abnormality. Our finding Results is close to a Turkish study [9], which found that 37.2% The study was performed on 105 patients of of thalassemia patients had pruritus. β‑thalassemia; female individuals comprised 52.4% while male individuals comprised 47.6% of patients The pathogenesis of pruritus is unknown. It might be Table 1. The duration of thalassemic disease ranged due to the release of histamine from tissue mast cells from 1 to 59 years (Table 2). secondary to stimulation by iron deposits in the skin [10]. The main skin disorders that were noticed in Dry skin is known as xerosis. The condition is decreasing order of frequency were pruritus (34.4%), characterized by pruritic, dry, cracked, and fissured [Downloaded free from http://www.mmj.eg.net on Thursday, January 14, 2021, IP: 156.204.184.20] Larynx in rheumatoid arthritis Gaber and Galal 269 Table 3 Distribution of skin diseases among enrolled patients (n=105) 34 and 53% of the evaluated patients [12], whereas Skin manifestation n (%) 24.8% of our patients reported this symptom. This Pruritus difference may be related to the prevailing climatic No 69 (65.7) conditions. Yes 36 (34.3) Xerosis In our study, 23.9% of the patients showed No 79 (75.2) pigmentary disorders. Hyperpigmentation is Yes 26 (24.8) generalized and particularly pronounced in the Scar sun‑exposed areas of the face and hands. Most of the No 94 (89.5) affected patients had dark brown hyperpigmentation. Yes 11 (10.5 Fekri et al. have documented a prevalence rate of Pigmentary disorders No 80 (76.2) 28% for skin pigmentation in their patients with Bronze color 1 (1.0) thalassemia [13].
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