Sickle Cell Ulcers: Classification and Clinical Features

Sickle cell ulcers are a common characteristic of an uncommon disease. Sickle cell disease predominantly affects black individuals, often having a seriously adverse effect on quality of life. Symptoms associated with sickle cell disease include ‘crisis’ episodes of fever and pain; severe abdominal pain; bone, joint, and back pain; and fertility problems. It has also been shown that 75% of sickle cell patients experience sickle cell ulcers over the course of their disease.1

Where do Sickle Cell Ulcers Occur?

Sickle cell ulcers occur commonly on the ankles and other areas of the leg where there is less subcutaneous fat, relatively thin skin, and a decreased blood flow. Sickle cell leg ulcers are painful and often disabling and tend to heal only slowly. Indeed, leg ulcers may be classified as acute or chronic according to their healing time.2 An acute ulcer usually heals within less than 1 month, while an ulcer that has not healed within 6 months is usually defined as chronic. It is not uncommon for chronic ulcers to last many years, often closing and re-opening repeatedly.

Characteristics of Sickle Cell Ulcers

Like other ulcers, sickle cell ulcers may be staged according to their depth and clinical characteristics. A stage 1 ulcer is characterized by non-blanchable erythema on intact skin, while a stage 4 ulcer involves full-thickness skin loss with extensive destruction, tissue necrosis or damage to muscle, bone or supporting structures.

Treatment of sickle cell ulcers can be challenging and frustrating, with very little randomized evidence currently available to direct treatment strategies. Because many sickle cell ulcers are difficult to treat,  recalcitrant, and the source of considerable incapacity, particular attention should be paid to guiding sickle cell patients in how to prevent the formation of sickle cell ulcers in the first place.  Prevention strategies include keeping the skin moisturized and avoiding trauma to the lower legs and ankles.

Although no clear guidelines exist for the management of sickle cell ulcers, some therapeutic strategies have been shown to offer some benefit. Next week we will look the pharmacological and wound-care interventions that are currently used.

Learn More About Sickle Cell Ulcers

 

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References

1. Charache S. One view of the pathogenesis of sickle cell diseases. Bull Eur Physiopathol Respi 1983; 19: 361-66.
2. Minniti CP, Eckman J, Sebastiani P et al. Leg ulcers in sickle cell disease Am J Hematol 2010; 85:831-833.
3. Treadwell TA, Willis AC, Brem H. Sickle Cell Ulcers. In: Baranoski S, Ayello EA, eds. Wound Care Essentials: Practice Principles. 2nd Edition. Lippincott Williams & Wilkins, Ambler PA. 2008