Sickle Cell Ulcer

A 39-year-old man who has sickle cell disease suffers with chronic ankle ulcers typical of the disorder. Ulcerations occur in approximately 50% of persons who are homozygous for sickle cell disease.

Drs Charles E. Crutchfield III and Humberto Gallego of St Paul comment that sickle cell disease is nearly unique to persons of African heritage. It is believed that the condition evolved to provide selective advantage in malaria-prone areas. In sickle cell carriers, red blood cells were less likely to be infected.

Skin ulcerations associated with this hemoglobinopathy usually begin in the second decade of life. The ulcers result from a partial vascular obstruction attributed to abnormal erythrocyte morphology (“sickle-shaped” cells) at sites of low local oxygen tension.

The ulcers present as sharply marginated, “punched-out” defects that are often located close to the malleoli. The lesions are painful, slow to heal, and prone to infection; they can be associated with joint deformity.

Management is primarily directed at prevention of the ulcerations. Once an ulcer appears, the leg needs to be elevated and immobilized, and the wound must be kept scrupulously clean. Transfusions, hyperbaric oxygenation, and dextranomer beads also have been used to promote healing.