Phenotypic characterisation and associations of leg ulcers in adult sickle cell patients

Abstract

Sickle leg ulcer (SLU) occurs as a result chronic occlusion of the vasculature with consequent necrosis of the skin and subcutaneous tissue usually in proximity of the malleoli. The description of clinical associations and the simultaneous occurrence of SLU and other complications of SCD compared to the non‐SLU patients was the aim of this work. A total of 272 (60.8% males and 39.2% females) patients were captured during this time period out of which 68 (51 males and 17 females) had SLU out of whom 20 patients had bilateral leg ulcers. Prevalence of SLU was 25% and the median age of patients was 25 years, frequency of crisis 2 per annum and 44 (74.6%) had been transfused in the past. Median Hb of the group was 7.6 g/dl and 25% had values lower than 6.5. The occurrence of other complications in SLU patients was as follows; 10 had AVN, 9 priapism, 8 had osteomyelitis, 6 nephropathy, stroke 2, osteoarthritis 4 and cholelithiasis 4. There was a significant relationship between the occurrence of SLU and gender of the patient being more in 67males—Likelihood ratio 4.610 (p = 0.032) and the occurrence of pulmonary hypertension—Likelihood ratio 4.762 (p = 0.029). There was no significant association between the occurrence of SLU and other complications of SCD. Leg ulcer patients have a median age of 25 years with a prevalence of 25% but do not necessarily show other features of severe disease phenotypes. SLU patients were more likely to develop pulmonary hypertension. Further studies on impact of environmental factors on the occurrence of SLU are needed to further evaluate its aetiology.

Controversies in the pathophysiology of leg ulcers in sickle cell disease

“…Many studies have shown an association between leg ulcers and echocardiographic signs of pulmonary hypertension. 13,19,68,69 Parent et al confirmed these results using right heart catheterization, 70 the gold standard for pulmonary hypertension diagnosis. These observations support systematic screening of pulmonary hypertension in SCD patients with leg ulcers.…”

“…Several studies reported a high incidence of leg ulcers in males compared to females with SCD. 11,13,[15][16][17][18] The male to female ratio was approximately 2 in the United States and Nigeria. 16,17 In contrast, other studies reported a similar incidence between males and females.…”

“…Several studies reported a high incidence of leg ulcers in males compared to females with SCD 11,13,15–18 . The male to female ratio was approximately 2 in the United States and Nigeria 16,17 .…”

“…Chronic leg ulceration in SCD has been associated with significant clinical and psychosocial morbidity 5–7 and an increased risk of early death 8 . Prevalence of chronic leg ulcers among individuals with SCD varies from 1.4% in Brazil to 29.5% in Jamaica 9–13 …”

“…8 Prevalence of chronic leg ulcers among individuals with SCD varies from 1.4% in Brazil to 29.5% in Jamaica. [9][10][11][12][13] In order to improve the management and care of SCD leg ulcers, the mechanisms behind their occurrence must be…”