Lymphedema Definition

Lymphedema is defined as “an accumulation of lymph fluid in the soft tissues, most frequently in the arms or legs.”^1,2 It impacts approximately one in every six patients in the United States who are undergoing solid tumor treatment. Lymphedema has become more prevalent with the increase in survival rates resulting from the emergence of more effective oncologic therapies; however, there remains no definitive cure for lymphedema.³ Lymph fluid is rich in protein and is filtered by the lymph nodes before being released into the bloodstream. If the lymph nodes become obstructed, lymph fluid backs up and the filtering system becomes overwhelmed, leading to collected fluid, which in turn causes swelling and edema. Lymphedema is a chronic condition and, if not appropriately managed, can result in irreversible skin changes, frequent infections, impaired mobility of affected extremity, and a reduction in quality of life.⁴ An article by Dayan et al, discusses research on lymphedema pathogenesis that indicated that the condition is fundamentally an immunologic process resulting in inflammation, fibroadipose deposition, impaired lymphangiogenesis, and dysfunctional lymphatic pumping.³ This research has also led to new medical and surgical therapies that offer more options in the plan of care than simply compression.³

Types of Lymphedema

There are two types of lymphedema. Primary—which can occur on its own—typically occurs in the legs but can affect the entire body. Primary lymphedema involves faulty or failed lymph node or blood vessel development. It is more frequently seen in women, it may or may not be present at birth or occur later in life, and although it is most often restricted to the lower extremities, it may affect the entire body. Secondary lymphedema stems from another disease or condition and is far more common than primary lymphedema. It is most often localized to the affected body part with absent or injured lymph nodes. Typically, secondary lymphedema is a result of surgical removal, injury, or destruction of lymph nodes or blood vessels. It can result as a complication of radiation treatment, it may be due to a tropical parasitic infection called lymphatic filariasis or elephantiasis, or it can develop as a result of chronic overload of the lymphatic system secondary to obesity, blood vessel problems, or recurrent skin infections.^1,5

Skin Care and Lymphedema

The skin is the body’s first line of defense against environment and external pathogens, and any opening or break in the skin provides a portal of entry for microoganisms. Lymphedema can lead to catastrophic and life-threatening skin breakdown. Complications of infections associated with lymphedema include, but are not limited to, cellulitis, lymphangitis, lymphadenitis, and ulcerations where protein-rich fluid provides a perfect medium for microbial growth.⁶ Education on the importance of good skin care is imperative for patients with lymphedema. Tips for skin care from Bryn Mawr Rehab Hospital include:⁷

• Reduce the risk of infection by avoiding trauma or injury.
• Keep extremities clean and dry, taking extra care to dry feet thoroughly, especially between toes.
• Apply emollients and moisturizers daily to prevent chafing and chapping, but steer clear of lotions with perfume or alcohol.
• Nail care should be provided by a podiatrist or nail care specialist.
• Extra care should be taken with razors to prevent nicks and cuts.
• Avoid punctures to the skin, such as injections and blood draws.
• Wear gloves during activities that increase the risk of skin injury, such as washing dishes, using chemicals, or using tools.
• If injury occurs, wash the area right away with soap and water, apply topical antimicrobial cream or ointment, and continue to observe carefully for signs of infection.
• A health care professional should be involved immediately if there is any rash, itching, redness, pain, increased skin temperature, increased swelling, fever, or flu-like symptoms.

Wound Care and Lymphedema

Because all cutaneous wounds are colonized with bacteria, the focus of skin and wound care for patients with lymphedema is on reducing bacterial bioburden. The body’s inflammatory response can result in extensive cutaneous changes leading to gross physical distortion of the limb. The skin can become so engorged that it weeps copious amounts of lymphatic fluid, so wound dressings must not only be highly absorbent but should also pull fluid away and lock it in to reduce skin and wound moisture and decrease the chance of maceration, which would lead to further skin breakdown and a decrease in epithelial migration from the wound periphery.

Copious amounts of drainage lead to the necessity for frequent dressing changes, resulting in expensive dressing costs, clinic visits, and lost time from work. It is estimated that the yearly cost of nursing and home care products for these patients can be in excess of $325,000.⁶ Bulky dressings may make it more difficult to apply compression devices correctly, so ultra-absorbent products should be used. This would also allow re-use of the compression garment if it is not ruined by drainage. Definitive compression garments typically cannot be prescribed until wounds are healed and edema is reduced. They must also be within the patient’s capability to don them and, of course, pay for them. Lack of coverage by Medicare or insurance for garments can lead to a revolving door of wounds and edema, then hospitalization and reduction, because these can cost hundreds to thousands of dollars.

Diagnosis of Lymphedema

The simplest diagnostic tool remains the Stemmer sign. It is demonstrated as a thickened fold of skin at the base of the second toe or second finger that can be gently pinched and lifted. Bioimpedance spectroscopy (BIS) is an electrical current passed through an extremity, after which impedance is measured. Reduced impedance is suggestive of lymphedema. Magnetic resonance imaging (MRI), computed tomography (CT) scan, and Doppler ultrasound may also help to identify where there is a potential blockage of the lymphatic system (such as from tumor growth). In lymphoscintigraphy, in which the movement of a radioactive dye through the lymphatic system is imaged, offers perhaps the most detailed view of the lymphatic system. The availability of these tests has improved; however, it is not always necessary to undergo these tests to treat lymphedema. If primary lymphedema is suspected, genetic testing is available to look for specific gene abnormalities.⁸

References

1. Kalra M. Lymphedema. Society for Vascular Surgery. 2019. https://vascular.org/print/patient-resources/vascular-conditions/lymphe…. Accessed May 17, 2019.

2. Rockson SG. General overview. In: Lee BB, Rockson SG, Bergan J, eds. Lymphedema: A Concise Compendium of Theory and Practice. New York, NY: Springer International Publishing; 2018:83–88.

3. Dayan JH, Ly CL, Kataru R, Mehrara BJ. Lymphedema: pathogenesis and novel therapies. Ann Rev Med. 2018;69(1):263–276.

4. Dylke S, Elizabeth L, Kilbreath S. Edema and lymphedema. In: MacLeod RD, van den Block L, eds. Textbook of Palliative Care. New York, NY: Springer International Publishing; 2018:1–11.

5. Mayo Clinic. Lymphedema. 2019. https://www.mayoclinic.org/diseases-conditions/lymphedema/symptoms-caus…. Accessed May 17, 2019.

6. Fife CE, Farrow W, Herbert AA, et al. Skin and wound care in lymphedema patients: a taxonomy, primer, and literature review. Adv Skin Wound Care. 2017;30(7):305–318.

7. Bryn Mawr Rehab Hospital. Ten skin care tips for individuals with lymphedema. Main Line Health. 2017. https://www.mainlinehealth.org/blog/2017/02/07/10-skin-care-tips-for-ly…. Accessed May 17, 2019

8. National Lymphedema Network. Position statement of the National Lymphedema Network. http://lymphnet.org/. Accessed May 20 , 2019.