Sickle Cell Information Center Guidelines

Edited by James Eckman, M.D. and Allan Platt, PA-C

Leg ulcers are common in hemoglobinopathy patients. They can be expected to develop in 25 to 100 % of patients with sickle cell anemia during their lifetime depending on their geographic location. The incidence of leg ulcers in the U.S. is about 10 per 100 patient years. . These are resistant to therapy, tend to be recurrent, and cause physical, psychological, and social disability. Prevention is the approach of choice and successful treatment requires a consistent, systematic approach to maximize patient compliance.

Present Illness. Document onset of ulcer, new, recurrent, ankle edema, trauma, pain, swelling, fever or chills. Determine the types and success of past treatments.

Past Medical History. List past surgery, present medications, and drug allergies.

- Vital Signs. Temperature

- Extremities. Note size of ulcer exactly, depth, presence and character of exudate, presence of granulation tissue, surrounding edema or erythema. Measure the size of the ulcer in two perpendicular directions if simple. Draw and measure all dimensions if complex.

- Lymphatics. Determine presence, size, and tenderness of inguinal and femoral nodes. Look for lymphangitis.

- Minimum Evaluation. CBC with differential and reticulocyte count. Culture if ulcer appears to be infected or cellulitis is present. Obtain x-rays if ulcer appears infected, leukocytosis with left shift is present, or patient is febrile.

- Additional Evaluation. For deep ulcers, systemic symptoms or intense pain with negative pain films of the area, bone scan or gallium scan may be indicated to exclude osteomyelitis under the ulcer. Blood cultures are indicated for patients with fever, neutrophilia, cellulitis, lymphadenitis, or osteomyelitis.

- Leg Ulcer. The presence of a leg ulcer is obvious by examination. Definitive characterization requires defining predisposing factors such as prolonged standing, trauma, previous home treatment, and edema. Complications such as osteomyelitis, cellulitis, and lymphadenitis must be excluded.

- Osteomyelitis. Fever or increased leukocytosis with left shift, fistula, deep pain favor this complication. Bone x-rays, bone scan, and gallium scan may be helpful in excluding infection of underlying bone, however, false positive results are frequent because of bone and flow changes caused by the ulcer. . X-rays are least sensitive, bone scan most sensitive, and gallium scan or indium labeled WBC scan may be more specific. Needle aspiration and biopsy may be necessary to establish or exclude osteomyelitis under an ulcer and to define the causative organism. There is generally great hesitation in performing such biopsies because of the potential to introduce infection into the bone from the ulceration.

- Cellulitis. Fever, increased leukocytosis with left shift, erythema or tenderness proximal to ulcer suggest this complication. X-ray and bone scan should be negative other than changes related to increased blood flow.

- Lymphadenitis. Fever and left shift with tender inguinal or femoral lymph nodes establish this diagnosis.

- Patients with osteomyelitis or lymphadenitis should be admitted for cultures and intravenous antibiotics. Treatment of large, necrotic, purulent ulcers may be facilitated by initial bed rest and frequent debridement in the hospital.

The treatment of leg ulcers starts simple and escalates in intensity if the ulcer fails to respond. Ulcers that have necrotic bases or eschar or purulent exudate are debrided with wet-to-dry, Duoderm, Mesalt, hydrotherapy, or surgical debridement. Once healthy granulation tissue is established, Unna boots, Duoderm, or wet-to-dry dressing promote healing. Transfusion therapy and skin grafts are reserved for large or resistant ulcers. All patients with leg ulcers should be started on zinc supplementation by giving zinc sulphate 200 mg. P.O. B.I.Dantibiotics are reserved for patients with cellulitis, lymphadenitis, and osteomyelitis. Topical antibiotics appear add little to management. All patients are instructed to elevated the leg as much as is possible and to use elastic support when on their feet for prolonged periods.

- Wet to Dry Dressings. Saline wet to dry dressings are applied T.I.Duse open, sterile 2 x 2 dressing, place it over the ulcer, saturate with sterile saline, let dry then pull off. If done properly, debris and necrotic tissue will be removed with each change. It is very important to pull the dressing off dry resisting the common tendency to wet the dressing for removal to reduce pain. Kling or dry gauze can be placed over the wet dressing loosely while drying is occurring to hold it in place. If the ulcer has considerable infected debris, whirl pool and concurrent surgical debridement may be indicated.

- Mesalt hypertonic saline dressings. Mesalt dressing are most useful on ulcers with considerable exudate. These cotton gauze dressings impregnated with crystalline sodium chloride are applied once or twice daily depending on the amount of exudate. The hypertonic environment that is created removes exudate and bacteria from the wound. These should be discontinued once debridement is accomplished and healthy granulation tissue is established for complete healing.

- Duoderm Dressing. This hydrocolloidal, oxygen impermeable dressing creates an environment over the ulcer that allows biologic debridement by normal phagocytic blood cells. Duoderm is useful for debridement of contaminated ulcers, facilitating healing when good granulation tissue is present, and protecting recently healed ulcers while the scar organizes. Pain is usually reduced by Duoderm making it a good choice for painful ulcers over other forms of initial therapy which usually increase pain. The dressing is changed at least once a week or when exudate starts to escape. The ulcer bed is gently irrigated with warm sterile saline keeping exposure time to a minimum. The ulcer is covered with new Duoderm providing at least a one inch margin around the entire ulcer. Deep ulcers are filled with Duoderm granules so the surface is level before applying the dressing. A gauze covering is taped over the dressing. Unna boot or elastic bandage may be applied for edema.

Considerable education is indicated when using Duoderm. The dressing will support complete debridement of non-viable tissue in and around the ulcer so the ulcer will usually grow in size before healing occurs. The patients must be made aware of this so that they do not loose confidence in the treatment. The wound will often smell funny or bad if it is leaking. Leaking is an indication for applying a new dressing and this may be required frequently during initiation of therapy. Large amounts of yellow fluid or gel are common and a good sign.

- Unna Boot. Unna boots are usefully in all patients with good granulation tissue. In patients with edema or those who like to put "things" on ulcers, the Unna boot may be the treatment of choice. The ulcer should have a relatively clean base before applying the boot or it should be changed in 3 - 4 days. Zinc oxide impregnated gauze is applied from the ankle to below the knee and then wrapped with a 4 inch elastic bandage. It must be smooth, fit snugly, and have decreasing tension proximally. The bandage will tighten as it dries. The boot is changed once a week until healing occurs. It is usually good to continue the dressing for several weeks after healing is complete.

- Hypertransfusion. Transfusion therapy appears to benefit some patients with ulcers resistant to more conservative measures. The goals of therapy are to maintain the hematocrit between 30 - 35 vol. % and the percentage of Hb A at > 70 % (See transfusion protocol).

- Skin Grafting. Patients with transfusion resistant or very large ulcers should be referred to plastic surgery for evaluation for skin grafting. Preparation includes transfusion and debridement with usual therapy until healthy granulation tissue is established. In general, the results of skin grafting have been disappointing with a high rate of graft failure and later recurrence of the ulcers. Grafts which include muscle can be useful in large, deep ulcers.

• Provide education to patient:
• Avoid injury especially to feet, ankles and legs
• To wear socks and appropriate fitting shoes
• Use insect repellants
• Avoid prolonged periods of time on feet
• Do not allow needle sticks to lower extremities
• Treat minor trauma promptly
• Do not smoke
• Teach patient treatment and protective measures
• Types of dressings and changing techniques
• Using aseptic technique when dressing wound
• Elevate legs as much as possible
• Eat a nutritious, well balance diet and take zinc supplements as ordered
• Reinforce that compliance with treatment is the most important determinant of successful healing
• Educate about judicious use of narcotics for this chronic pain and alternative pain control methods
• Teach patient to avoid trauma, keep skin lubricated, leg as often as possible and to wear elastic support hose after the ulcer area heals to prevent recurrence
• Provide counseling if patient is experiencing self/body image issues interfering with therapy

Prevention of leg ulcers is very important. Patients must be instructed to treat minor trauma around the ankles promptly. Insect repellants and protection from chiggers is important. Edema should be treated with elevation of the legs or elastic stockings.

Once the ulcer heals, the patient should be instructed to avoid trauma to area, lubricate the area with skin moisturizers, and treat edema with leg elevation and elastic support. In our experience, edema is the single most common antecedent to recurrence of a leg ulcer.

Prevention of ulcers must be stressed beginning in childhood. The patient undergoing treatment must be given complete instructions and education about the normal course of treatment. The long-term nature of the ulcers and the importance of consistent and regular care must be stressed. Painful ulcers require education about judicious use of narcotic analgesics for chronic pain. Once the ulcer is healed all of the method of prevention must be reinforced with each visit.

Chernoff AI, Shapleigh JB, and Moore CV. Therapy of chronic ulceration of the legs associated with sickle cell anemia. J.A.M.A. 155(17):1487-1491, 1954.

Wolfort FG and Krizek TJ. Skin ulceration in sickle cell anemia. Plast. & Reconstr. Surg. 43(1):71-77, 1969.

Serjeant GR, Galloway RE, and Gueri MC. Oral zinc sulphate in sickle-cell ulcers. Lancet. 2:891-893, 1970.

Serjeant GR. Leg ulceration in sickle cell anemia. Arch. Intern. Med. 133:690-694, 1974.

Alleyne SI, Wint E, and Serjeant GR. Social effects of leg ulceration in sickle cell anemia. South. Med. J. 70(2):213-214, 1977.

Braum KF, McFarlane DE, Maude GH, and Serjeant GR. Topical antibiotics in chronic sickle cell leg ulcers. Trans. R. Soc. Trop. Med. Hyg. 81(5):847-849, 1987.

Ademiluyi SA, Rotimi VO, Coker AO, Banjo TO, and Akinyanju O. The anarobic and aerobic bacterial flora of leg ulcers in patients with sickle cell disease. J Infect 17:115-120, 1988.

Koshey M, Entsuah R, Koranda A, Johnson R, Bellvue R, Flounoy-Gill Z, and Levy P. Leg ulcers in patients with sickle cell disease. Blood 74:1403-1408, 1989.

Rubin JR, Alexander J, Plecha EJ, and Marman C. Unna’s boot vs polyurethane foam dressings for the treatment of venous ulceration. Arch. Srug. 125:489-490, 1990.

Phillips G, Eckman JR, Hebbel RP. Leg ulcers and myofascial syndromes Trait in Sickle Cell Disease: Basic Principles and Clinical Practice Embury SH, Hebbel RP, Mohandas N, Steinberg MH eds. Raven Press NewYork 1994. pp.681-688.

Eckman JR. Leg ulcers in sickle cell disease. Hematol Oncol Clin North America !0:1333-1344, 1996.

Ohene-Frempong K Steinberg MH. Clinical aspects of sickle cell anemia in adults and children. in Disorders of Hemoglobin: Genetics, Pathophysiology and Clinical Management. Steinberg MH, Forget BG, Higgs DR, Nagel RL. Cambridge University Press Cambridge UK 2001. pp. 642-644.

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Copyright © 1997 Sickle Cell Information Center
Last modified: June 12, 2001