Leg Ulcers in Sickle-Cell Disease: Treatment Update

Abstract

Sickle-cell leg ulcers (SCLUs) are a severe, chronic, and recurrent complication of sickle-cell disease (SCD). There are no official recommendations for treatment. Recent Advances: Only a few studies with a high level of evidence have been conducted to evaluate treatment of SCLUs. However, several studies have been conducted with a high level of evidence to evaluate the efficacy of treatments in venous leg ulcers, and SCLUs could benefit from these treatments, especially when a venous incompetence or an edema is associated. Pathophysiology of SCLUs includes a vasculopathy related to chronic hemolysis and an endothelial dysfunction, which could be therapeutic approaches to SCLU treatment. Critical Issues: Therapeutic approaches to SCLUs can target SCD on the one hand and skin healing and associated aggravating factors on the other. A review of the literature found only case series and six randomized controlled trials; some offered encouraging results, but most had serious biases. Clinical trials specifically targeting SCLUs are difficult to realize because of the small number of affected patients, in comparison with patients with leg ulcers from other causes. Future Direction: Treating SCLUs remains a challenge. Data in the literature are currently insufficient to offer clear treatment guidelines because of several biases in controlled studies. New studies are under way to assess the efficacy of topical treatments and describe the microbiome of SCLUs. Prevention of SCLU recurrence should be assessed in future clinical trials because the high risk of recurrence is an unsolved critical issue.

Enhanced Hypercoagulability in Sickle Cell Anaemia Patients with Chronic Leg Ulcers

“…Also, SCA patients with CLU in our study had significantly lower Hb concentration compared to those without CLU. This supports the earlier finding associating CLU in SCA with haemolysis and severe anaemia [ 8 ].…”

“…Due to the chronic nature of these ulcers, SCA patients with CLUs may experience significant disfigurement, social isolation, and loss of income [ 3 ]. The increased disability, absence from work or school, and high utilization of healthcare resources severely affect the quality of life of individuals with SCA and CLUs [ 8 , 9 ]. Earlier, Ankra-Badu (1992) reported the mean duration of a sickle cell CLU in Ghana as three and half years [ 10 ], a more recent study obtained a mean duration of 4.6 years [ 5 ].…”

Profile of Women With Leg Ulcers Due to Sickle Cell Disease

“…This fact is probably due to the suspicion of hydroxyurea causing leg ulcers in patients with SCD. However, this side effect remains controversial 23 , including in a systematic review of the literature, hydroxyurea treatment of people with SCD was not significantly associated with an increase in the occurrence of leg ulcers 22 .…”

Recomendações para o manejo da úlcera da perna em pessoas com doença falciforme

“…Sugere, também, a utilização de oxigênio hiperbárico e terapia a vácuo. Destaca-se que uma meta-análise recente não revelou benefício da oxigenoterapia hiperbárica em feridas crônicas 27 , porém há apenas relatos de casos da sua utilização em úlceras decorrentes da DF 7,28 . Quanto à terapia a vácuo, não foi identificada literatura sobre a sua utilização nas úlceras decorrente da DF 28 .…”