|Year : 2014 | Volume
| Issue : 3 | Page : 141-145
Chronic leg ulcers in sickle cell disease patients in Zaria, Nigeria
Abdulaziz Hassan1, Dogara L Gayus1, Ibrahim Abdulrasheed2, Musa A Umar1, Dahiru L Ismail3, Ahmadu A Babadoko1
1 Department of Haematology, Ahmadu Bello University Teaching Hospital, Shika, Zaria, Nigeria
2 Department of Surgery, Ahmadu Bello University Teaching Hospital, Shika, Zaria, Nigeria
3 Department of Orthopaedics, Ahmadu Bello University Teaching Hospital, Shika, Nigeria
|Date of Web Publication||8-Dec-2014|
Dr. Abdulaziz Hassan
Department of Haematology, ABUTH PMB 06, Shika - 810 001 Zaria
Source of Support: None, Conflict of Interest: None
Background: Chronic Leg Ulcers (CLU) are common cutaneous manifestation of sickle cell disease (SCD) and a major cause of debility. CLU results from vaso-occlusion, poor venous pressure, hypercoagulabilty and trauma. The incidence of CLU is variable based on geographical region. Treatment modalities include debridement and skin grafting. The aim of this study was to determine the prevalence, incidence, hematological parameters and treatment modalities of CLU among SCD patients in Ahmadu Bello University Teaching Hospital (ABUTH) Zaria.
Patients and Methods: It was a retrospective study of adult SCD patients between January-December 2012 at ABUTH Zaria. Case notes of SCD patients were retrieved and data on socio-demographics, hematological parameters and CLU were extracted and analyzed using Epi Info. Data was analysed using descriptive analysis and comparisom of mean by student's t-test.
Results: Of the 446 SCD patients attended to during the study period 14 had CLU. The incidence of CLU was 0.45% among the SCD patients studied. The male to female ratio was (M: F = 1.3:1). CLU occurs commonly around the malleoli in 78.6% whereas other sites constituted 21.4%. In 77.8% of CLU low steady state PCV was seen, 76.9% had reticulocytosis whereas 81.8% had high platelet count > 477 × 109/l. Recurrence rate was 78.5% with osteomyelitis (16.7%) and limitation of movement (16.7%) as a frequent complications of CLU. Wound debridement and dressing was the commonest surgical management option but with a high recurrence rate.
Conclusion: Prevalence of CLU is low in SCD patients in Zaria; occurring commonly in patients with low steady state PCV, reticulocytosis and thrombocytosis. Debridement and skin grafting has good cure rate and lower recurrence of CLU.
Keywords: Chronic leg ulcer, sickle cell disease, skin graft
|How to cite this article:|
Hassan A, Gayus DL, Abdulrasheed I, Umar MA, Ismail DL, Babadoko AA. Chronic leg ulcers in sickle cell disease patients in Zaria, Nigeria. Arch Int Surg 2014;4:141-5
|How to cite this URL:|
Hassan A, Gayus DL, Abdulrasheed I, Umar MA, Ismail DL, Babadoko AA. Chronic leg ulcers in sickle cell disease patients in Zaria, Nigeria. Arch Int Surg [serial online] 2014 [cited 2019 Jun 25];4:141-5. Available from: http://www.archintsurg.org/text.asp?2014/4/3/141/146405
| Introduction|| |
Sickle cell disease (SCD) is a collective term for a group of blood diseases resulting from the inheritance of the defective hemoglobin S.  Homozygous SCD has a worldwide frequency of 2-38%.  It is estimated to be 8% in blacks in America, 8% in blacks in Jamaica and 40% in tropical Africa.  The frequency of sickle cell trait in equatorial Africa is 10-30%, in Nigeria it is estimated at 25%. , The World Health Organization (WHO) recognizes SCD as a problem of major public-health significance. ,, The manifestations of this disease are protean.  Chronic complications include cerebral syndrome, priapism, chronic organ damage, osteonecrosis, renal failure, chronic hemolytic anemia and Chronic Leg Ulcers (CLU). ,
These ulcers are characterized by an indolent, intractable course with severe consequences of pain, physical disfigurement and high utilization of health care resources. , It often leads to social isolation and psychological disturbances that may result in retardation of educational progress and diminished prospects for employment. , Although rarely contributing to death, they constitute in their prolonged form a severely disabling condition because they are resistant to therapy and are recurrent. A patient who experiences a first ulcer is likely to ulcerate again. Approximately 97% of healed sickle cell ulcers will recur in less than 1 year. History of a sickle cell leg ulcer carries a 23-fold increased risk of developing future ulcerations; having one active ulcer carries a 146-fold increased risk. ,,,,
The pathogenesis of CLU is poorly understood but the consensus is that it is multifactorial.  However, the three major factors are marginal blood supply to the skin, local edema and trauma.  Tissue injury due to mechanical obstruction of vessels by sickle cells and upregulation of adhesion molecules such as integrins on the endothelial cells results in platelet aggregation. Increased interactions between granulocytes and sickled red cells, release of injurious cytokines,  increased blood viscosity in cases of thrombocytosis or dehydration, deficiency of antithrombin III which promotes vascular thrombosis , and increase circulating neocytes and nucleated red cells have all been implicated in the pathogenesis of CLU.
The aim of this study was to determine the incidence, characteristics and management modalities of CLU among patients with SCD in Zaria, North-West Nigeria.
| Patients and Methods|| |
It was a retrospective study involving review of both old and new SCD patients' record at the sickle cell clinic, hematology day care clinic, plastic surgery clinic and accident and emergency unit of Ahmadu Bello University Teaching Hospital (ABUTH) Zaria over a year (January to December 2012). Ethical approval was obtained from the ABUTH Zaria Health Research Ethics Committee. Using a profoma, the following data were extracted; socio-demographics (age, sex and socioeconomic status) steady state hematological parameters (hematocrit, platelet count, white blood cell count and reticulocyte count) and CLU (etiology, duration, anatomical site, size and treatment modality).
The data obtained was entered into Epi Info software version 3.5.3. and analyzed. Student's t-test was employed to compare means of hematological parameters between SCD patients with CLU and those without CLU. The level of significance was set at P < 0.05.
| Results|| |
Of the 446 SCA patients attended to during the study period only two (0.45%) were sickle-hemoglobin C (HbSC). A total of 14 had CLU giving a prevalence of 3.1% out of which only 2 were new cases giving an incidence of 0.45%, all the 14 SCD patients with CLU were sickle-hemoglobin S (HbSS). The mean age of patients with CLU was 28.3 years with a range of 19-40 years. Among those with CLU, there were eight males and six females (M:F ratio of 1.3:1) and 10 (71%) were of low socioeconomic background as depicted in [Table 1].
[Table 2] highlights the causes and duration of CLU which were spontaneous and of moderate duration (7-18 months) in 7 (50%) and 11 (79%) of the cases respectively. Similarly, CLU was found most commonly on the right leg only, malleoli and of 6-10 cm size occurring in 7 (50%), 12 (86%), and 9 (64%) of the cases respectively. Modality of treatment were debridement and dressing was the commoner modality of treatment as it was used in 8 (57%), however it had a higher recurrence rate of 5 in 8 (63%) as shown in [Table 3]. Osteomyelitis was found in 2 (16.7%) and limitation of movement in another 2 (16.7%).
|Table 2: Aetiological and physical characteristics of CLU among SCA patients in Zaria|
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|Table 3: Treatment option and recurrence of CLU in SCA patients in Zaria|
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The mean hematological parameters in the steady state without CLU versus those with CLU were as outlined in [Table 4]. It is worthy of note that most 10/14 (78.6%) patients with CLU had steady state packed cell volume (PCV) lower than the mean PCV for the group. In contrast those without CLU about 57.1% had steady state PCV above the mean for the group. 13/14 (92.9%) patients with CLU have reticulocytosis above 10%, while those without CLU have a mean reticulocyte count of 8.4%.
| Discussion|| |
CLU has been a long recognized complication of SCD and were present in the first four reported patients with sickle cell disease. , The prevalence and nature of CLU in SCA patients varies across the world due to variation in genotype, phenotype and geographical location. The prevalence of CLU among adults with SCA in Zaria North-West Nigeria was 3.1% from this study. This incidence is lower than the 9.6% and 7.5% reported in South-south and South-west geographical zones of Nigeria respectively. , This discrepancy is likely due to the difference in geographical location between the Northern and Southern geographical zones of Nigeria. This prevalence is also lower than 13.2% and 10.6% for Sierra-Leone and Ghana for the same reasons of difference in geographical location. 
The finding of a lower mean steady state PCV in majority of patients with CLU is in agreement with Bazuaye et al., who reported CLU in patients with PCV of 18.3-24% in 58.4% in their patients in Benin South-South Nigeria.  Low steady state PCV and reticulocytosis are markers of hemolysis as described by Nolan et al.  The platelet count was higher among patients with CLU than in those without CLU even though it was not statistically significant. This is similar to the report by Madu et al., from Enugu in South-East Nigeria. 
All the patients with CLU in this study were HbSS which further confirms the fact that CLU is commoner in patients with HbSS and rare in those with HbSC.  The mean age of patients with CLU was 28.3 years this indicating the rarity of CLU below 10 years of age and increasing incidence from 10-20 years of age.  The mean age is also similar to reports from South-West Nigeria  and most researchers agreed that leg ulcers are most commonly reported in adolescence and early adulthood in individuals with SCD.  A similar age pattern was reported by the Cooperative Study of Sickle Cell Disease (CSSCD) in the United States, although the maximum incidence occurred slightly later: between 20-50 years. 
The male to female ratio indicates a slight male preponderance in this study. A male preponderance has been reported by other workers from Ghana.  However, no gender difference occurred in the Jamaican Cohort Study.  This disparity suggests that environmental factors rather than genetic predisposition may explain the sex differences. 
SCA patients from a low socioeconomic status had the highest incidence of CLU. These are followed by the medium and high status group and there was a negative correlation between socioeconomic status and incidence of CLU (r = −0.95). School dropouts and subsequently unemployed or underemployed SCA patients have been reported to have high incidence of CLU. ,
The history of trauma in 21% of the patients with CLU in this study is lower than the 40% reported in by Ankra-Badu.  This finding may be as a result of insect bites, itching, trauma from scratching, or the use of the ankle or foot as site for intravenous therapy or vascular access in patients with difficult veins have been suggested as etiological factors.  Similarly, the warm tropical climate and high humidity may have favored the persistence of skin lesions.  Thus, these factors and a low socioeconomic status have a major influence on development of CLU. 
The major site affected was the skin around the malleoli (86%) and the right leg was more commonly affected than the left leg. The CSSCD in the USA found no specific site predeliction.  Our finding may be due to CLU occurring in areas with less subcutaneous fat, thin skin, and decreased blood flow.
The management of sickle cell leg ulcers aims at effectively healing the lesion and thus improving the quality of life.  In this study, 57% of the CLU healed with wound debridement and povidone iodine dressing. This is similar to the study by Idaewor et al.,  where routine dressings with antibiotic powder appeared more likely to improve ulcer healing than surgical approaches. , Several authors report that with regular and good localized treatment, many small CLU in SCA heal and heal faster than other types of leg ulcers. ,
Split thickness skin graft after debridement was done for 43% of the CLU patients in this study but has a recurrence rate of 50%. This was similar to a recurrence rate of 52% in a multicentre study by Koshy et al.  The high failure rate after skin grafts may be due to large diameter ulcers, and longer duration of the ulcer before surgery. Similarly Trent et al.,  suggested that most of the grafts may fail because of the inherent circulatory difficulties and vascular insufficiency in the areas of these ulcers. Thus Jamaican researchers suggests that ulcers must be completely healed before discharge from hospital but recurrence rates were between 80-97% within 2 years even after successful graft.  Recently, Oliveira et al.,  reported a successful outcome with negative pressure therapy (NPT) in preparation of the wound bed for skin graft in a 28-year-old woman with multiple CLU. The granulation tissue had an even surface, which resulted in a cleaner and reddish healthy-looking wound bed. The skin grafts on the NPT-treated wounds remained resilient, pliable and there was no recurrence 11 months after surgery. This encouraging result suggests that research to explore the safety and effectiveness of NPT in patients with sickle-cell leg ulcers is warranted.
The prevention and active treatment of early sickle cell leg ulcers may prevent the development of extensive fibrosis as well as the healing - relapsing course of large lesions.  Measures such as wearing properly fitting shoes and promptly treating any minor trauma are essential components of prevention. Other important prevention strategies include proper hygiene, liberal use of emollients to reduce drying and cracking of the skin and providing firm supporting bandages to reduce stasis and lymphoedema if the patient is ambulant. , However, adherence to treatment is not always effective, especially considering the socioeconomic status most of the patients and the time required for therapy. 
| Conclusion|| |
CLU is common among patients with SCA in Zaria. Low socioeconomic status, low steady state PCV and increased reticulocyte count are commoner in those with CLU. Healing rates are higher with debridement and skin grafting than debridement alone though both have risk of recurrence. There is a need for patient education on preventive measures to reduce incidence of CLU among SCA patients in Zaria, Nigeria.
| Acknowledgements|| |
1. Mrs Lami N. KATO - Nursing Officer who helped in tracing pacients records.
2. Dr. Rasheed YUSUF - Who helped with statistical analysis and data presentation.
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[Table 1], [Table 2], [Table 3], [Table 4]