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Managing chronic complications of sickle cell disease: leg ulcers

Leg ulcers are a common complication of sickle cell disease ( SCD ) in general and sickle-cell anaemia ( SCA ) in particular. Leg ulceration was reported in all of the first four people with sickle cell disease described in the English literature.
Data from the Cooperative Study of Sickle Cell Disease ( CSSCD ) in the United States found active leg ulcers at entry in 2.5% of 2,075 people aged 10 years or older and in none of 1,700 people less than 10 years old. Among those with active leg ulcers, about 22% were between the ages of 10 and 20.

Data on leg ulcers from the CSSCD321 identified five factors which could affect the person’s risk.
Leg ulcers were more common in males and older people and less common in people with alpha-gene deletion, high total Hb level, and high levels of HbF.
Trauma, infection, and severe anemia may predispose people to ulcer formation.
Studies showing a positive association between leg ulcers and the severity of hemolysis and priapism are disputed.

The ulcers occur most frequently on the medial or lateral surfaces of the ankles. Leg ulcers can range from mild and small to large and severe. Severity can be based on depth and duration.

Osteomyelitis may complicate chronic leg ulcers, especially deeper ones. A bone scan or MRI and bone biopsy are used to assess this complication.

Multidisciplinary teams including wound care specialists have been developed to provide support and consultation in the management of recurrent and recalcitrant leg ulcers. ( Xagena )

NIH - Evidence-Based Management of Sickle Cell Disease - Expert Panel Report, 2014