Diagnosing Repeated Lower Extremity Ulcerations
A patient’s medical history can provide you with critical information that is necessary to differentiate among the various types of lower extremity ulcerations. Not only are there many different etiologies of lower extremity ulcers, it’s also important to be aware of the patient’s underlying medical conditions as well, as you will see in the following case study. A 38-year-old Caucasian female patient recounted her underlying medical conditions when she came in for evaluation and treatment of her left leg ulceration. She injured the side of her leg while walking and had the ulceration for about a month. She noted that she has had ulcers in this area in the past. The patient says the last time she had the same exact ulceration, it took a little over a year to heal. Her past medical history is significant for osteoporosis, rheumatoid arthritis, microcytic anemia and lower extremity edema. She also noted that she had many blood transfusions when she was in her 20s. This patient is young and very active, which makes it difficult for her to remain off her feet. She explained that the more swelling she has in the lower limb, the more pain she feels. What Is The Diagnosis? 1. Sickle cell anemia 2. Rheumatoid arthritis 3. Thalassemia 4. Pyoderma gangrenosum Possible Answers 1. Sickle cell anemia is not the correct answer. Sickle cell anemia is a chronic hemolytic anemia occurring almost exclusively in African-Americans. It is characterized by sickle-shaped red blood cells caused by homozygous inheritance of Hb S. With the sickling and capillary plugging with deformed red blood cells, the surrounding tissue becomes ischemic with tissue necrosis and ulceration occurs. You’ll usually see these sickle cell ulcers below the knee. They are usually chronic, shallow and develop in relation to a sickle cell crisis. You’ll notice a typical “punched-out” appearance with well-defined wound edges. The tissue bed is usually granulating. Keep in mind that these ulcers usually appear in patients with more severe anemia. Debridement of any necrotic tissue and maintaining a moist wound environment should enhance wound healing. In some cases, using an Unna boot may be effective to promote wound closure. If all other treatments have failed, you may try to perform skin grafting. Also be aware that when a patient is having a sickle cell crisis localized to the extremities, it may mimic osteomyelitis, acute arthritis (such as gout) or rheumatoid arthritis. 2. Rheumatoid arthritis is not the correct answer. It is a chronic, multi-system disease of unknown etiology. The characteristic feature of rheumatoid arthritis is persistent inflammatory synovitis. The peripheral joints are involved in a symmetric distribution. Patients with severe manifestations of rheumatoid arthritis may also suffer from rheumatoid vasculitis, which can affect any organ system in these patients. In its most aggressive form, rheumatoid vasculitis can cause polyneuropathy, cutaneous ulceration, dermal necrosis and digital gangrene. Large ischemic ulcerations develop in the lower extremity. The pathogenesis of these types of ulcers is not well understood. These particular ulcers may take a long time to heal. (Also keep in mind that Felty’s syndrome characteristically comprises rheumatoid arthritis, leukopenia and splenomegaly. About one-quarter of these patients are affected by large refractory skin ulcerations.) Vasculitic ulcers are often associated with infiltration and inflammation of small vessels in the skin. These ulcers are most often associated with patients who have inflammatory conditions such as rheumatoid arthritis, scleroderma and systemic lupus erythematosus. Characteristically, these ulcers are painful and may be multiple and confluent. A biopsy may be necessary to confirm the diagnosis. Small vessel vasculitis accounts for about 10 to 20 percent of lower leg ulcerations in rheumatoid arthritis. Prednisone in combination with methotrexate has been proven successful in managing rheumatoid vasculitis. 3. Thalassemia is the correct answer. Thalassemias are a group of chronic inherited microcytic anemias characterized by defective Hb synthesis and ineffective erythropoiesis. Thalassemia is more common in people of Mediterranean, African and Southeast Asian ancestry. This ulcer did reoccur after a minor injury to the affected area. 4. Pyoderma gangrenosum (PG) is not the correct answer. It is a noninfectious destructive inflammatory skin disease that leads to a very painful large ulceration with an undermining violaceous pustular border. These ulcers often begin as pustules and then they expand rapidly to a larger size. They are often described as rapidly enlarging, purulent and usually present with undermined bluish borders. Other ulcerations may develop from inflammatory nodules or from a trauma to normal-appearing skin. These patients are very prone to the development of additional pyoderma gangrenosum ulcers. Therefore, you should avoid any surgical debridement. The types of ulcers are usually very painful and have a propensity for the lower extremity and trunk areas. An enlarging ulcer will usually present with an intense erythema around the wound. While you’ll usually see these types of ulcers on the lower extremity, they can arise anywhere on the surface of the body. Approximately 30 to 50 percent of these cases are idiopathic and the most commonly associated disorders are ulcerative colitis and Crohn’s disease. It is less commonly associated with chronic active hepatitis, myeloma, polycythemia vera and seropositive rheumatoid arthritis. The diagnosis of pyoderma gangenosum is often made clinically because the histology is often nonspecific. While the histology is not pathognomonic, neutrophilic infiltration and thrombosis of the small cutaneous vessels have been found in the borders of these types of ulcerations. Diagnosis And Treatment Systemic corticosteroids are the best recommended treatment for this condition. Additionally, cyclosporine has been shown to be effective in cases that have not responded to steroids. Chronic indolent ulcers near the malleoli have been described in many hemolytic disorders. Five percent of patients with thalassemia minor will develop a leg ulcer, which actually may be the first sign of the disease, as opposed to thalassemia major, in which leg ulcerations are uncommon. These types of ulcers are very similar to the sickle cell anemia ulcer and are typically located on the lower leg above the lateral malleoli. These types of ulcers present with a punched-out appearance, granulating tissue bed and well-defined margins. Thalassemia is among the most common inherited hemolytic disorders. Thalassemia results from unbalanced Hb synthesis caused by decreased production of at least one globin polypeptide chain. The clinical features of thalassemias are similar but will vary in severity. B-Thalassemia minor is clinically asymptomatic. B-Thalassemia major (Cooley’s anemia) presents with symptoms of severe anemia. These patients usually have jaundiced skin and present with leg ulcers and cholelithiasis. In Conclusion Always remember that a moist wound environment is more conducive to wound healing than a dry environment. In a moist wound environment, dermal wound repair is stimulated and epithelialization is accelerated. It’s also essential to ensure proper medical management of any underlying medical conditions your patient may have. The team approach can facilitate the treatment of these underlying disease processes and improve the quality of life for the patient. Once an ulcer has healed, it’s also pivotal to educate the patient about ulcer prevention. Dr. Fishman is Chairman of the Wound Care Institute in North Miami Beach, Fla.