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Lower extremity lymphedema: Update: Pathophysiology, diagnosis, and treatment guidelines

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Lower extremity lymphedema is an important medical issue which causes morbidity and is frequently seen by dermatologists. The subject has not been adequately addressed in dermatologic literature for many years. Primary lymphedema is caused by an inherent malfunction of the lymph-carrying channel, in which no direct outside cause can be found. Secondary lymphedema is caused by an outside force, such as tumors, scar tissue after radiation, or removal of lymph nodes, which results in dysfunction of the lymph-carrying channels. Treatment is based on rerouting the lymph fluid through remaining functional lymph vessels. This is accomplished through elevation, exercises, compression garments/devices, manual lymph drainage, and treatment is combined with good skin care practices.

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Etiology and pathophysiology of lower extremity lymphedema

Dermatologists frequently encounter patients with lymphedema of the lower extremities, often caused by chronic venous insufficiency and recurrent cellulitis. Although this is a relatively common problem, it has rarely been addressed in the dermatology literature. In this article, we will provide a concise review on definitions, causes, diagnosis, and treatment strategies in lymphedema.

Clinical presentation

Initially, patients with lower extremity lymphedema present with unilateral painless swelling that starts on the dorsal aspect of the foot, with eventual proximal involvement over the first year (Fig 2). The foot often has a squared-off appearance. The edema is initially a pitting edema, but over time the subcutaneous tissue becomes fibrotic, resulting in nonpitting brawny edema. The edema can then spread circumferentially if treatment is not initiated. Eventually, the involved skin becomes

Differential diagnosis

Lymphedema should be considered with any edematous extremity without pain or inflammation. Chronic venous insufficiency can be difficult to differentiate from early lymphedema because both have pitting edema, and skin changes typical of late-stage lymphedema are not yet present. Additionally, chronic venous insufficiency is often bilateral, rather than unilateral as in lymphedema. Lymphoscintigraphy may be necessary to distinguish the two, although the distinction cannot always be made since

Diagnosis

Whenever there is doubt regarding the clinical diagnosis, diagnostic confirmation can be accomplished with isotopic lymphoscintigraphy (considered the method of choice) or, if necessary, with radiocontrast lymphangiography.15 Computed tomographic (CT) scanning or magnetic resonance imaging (MRI) (MRI is superior to CT scans because it can detect water) of the lower extremity can also detect a “honeycomb” pattern of the subcutaneous tissue that is not characteristic of other types of edema.

Management

Lymphedema is associated with significant morbidity in terms of the functional, cosmetic, and emotional consequences of this chronic and potentially disabling condition. Treatment efforts are focused on minimizing the associated swelling, restoring cosmesis and functionality of the limb, and preventing potential complications associated with lymphedema (eg, cellulitis, lymphangitis). Treatments are time consuming, expensive, and involve a multidisciplinary approach among rehabilitative

Multidisciplinary treatment approach

The diagnosis of lymphedema is one with a chronic, disabling connotation. Through the joint efforts of a multidisciplinary team of lymphedema therapists, dermatologists, nurses, and occasionally vascular surgeons, a comprehensive treatment plan can be developed (see Fig 5). A description of each type of therapy should be presented to the patient with lymphedema, and a recommendation of an overall treatment plan should be discussed in detail.

There is a lack of high-quality randomized, controlled

References (34)

  • A. Tiwari et al.

    Differential diagnosis, investigation, and current treatment of lower limb lymphedema

    Arch Surg

    (2003)
  • M.E. Baldwin et al.

    Molecular control of lymphangiogenesis

    Bioessays

    (2002)
  • P.S. Mortimer

    ABC of arterial and venous disease: swollen lower limb-2: lymphoedema

    Br Med J

    (2000)
  • H.R. Durr et al.

    Stewart-Treves syndrome as a rare complication of a hereditary lymphedema

    Vasa

    (2004)
  • T. Nakazono et al.

    Angiosarcoma associated with chronic lymphedema (Stewart-Treves syndrome) of the leg: MR imaging

    Skeletal Radiol

    (2000)
  • P.S. Mortimer

    The pathophysiology of lymphedema

    Cancer

    (1998)
  • B. Danz et al.

    Radiotherapy in multilocalized lymphedema-associated angiosarcoma

    Eur J Dermatol

    (2005)
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    Funding sources: None.

    Conflicts of interest: None declared.

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