An 18-year-old woman seeks an opinion about swelling in her right leg. She first noticed it 1 year ago when her pants seemed to be tighter on that leg. She has no pain or discomfort; the edema does not improve after nocturnal rest.

On physical examination, the left leg is normal, and on the right the edema involves the toes, which look squared, along with nonpitting edema in the foot. The skin appears thickened, and there is a 4-cm difference in diameter between the legs measured in the calf. She has no adenopathy, and the abdominal examination is unremarkable. A diagnosis of lymphedema tarda is rendered.

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  Lymphedema is an accumulation of lymphatic fluid in the interstitial tissues, most commonly in the limbs, resulting in edema.

  
  
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  Incidence: primary lymphedema—1:6000 to 1:10,000; secondary lymphedema—varies among different populations; it can be as high as 30% in breast cancer patients after surgery and radiation therapy.^1,2

  
  
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  Primary lymphedema is more prevalent in females, 2.5 to 10:1.²

  
  
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  Lymphedema can affect patients from birth (congenital lymphedema) to advanced age.

  
  
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  Lymphedema rarely leads to death, but is a source of morbidity and affects body image.

  
  
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  The natural history varies from minimal swelling to severe deformities in the limb.¹

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  Lymphatic vessels collect and drain the excessive interstitial fluid that escapes the capillary circulation. Lymphedema is the accumulation of this protein-rich lymphatic fluid in the tissues.

  
  
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  Lymphedema occurs when the production of lymphatic fluid exceeds the transportation capacity of the lymphatic vessels.

  
  
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  It can affect multiple areas, but most commonly involves the upper or lower extremities.

  
  
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  In primary lymphedema there is an inherited defect in the lymphatic vessels that manifests at birth to the early 40s. Lymphedema can be the only manifestation, or it can be part of a syndrome (eg, yellow nail syndrome, lymphedema-distichiasis syndrome, Turner syndrome).²

  
  
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  Secondary lymphedema is more common, caused by destruction or damage of the lymphatics or increased production of lymphatic fluid. Common causes include filarial infection (in tropical and subtropical regions of Asia, Africa, and Central and South America),³ malignancy (involving lymph nodes or lymphatics), trauma (surgical damage or resection of lymphatics), chronic venous insufficiency (increased interstitial fluid), inflammatory diseases, recurrent bacterial infections, and chronic edema.^1,2

History

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  Lymphedema may present as unilateral, painless swelling with minimal change with elevation.

  
  
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  The swelling usually starts distally and is more noticeable in fingers or toes.

  
  
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  Recurrent cellulitis is a common complication.

  
  
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  History of trauma, surgery, radiation therapy, and malignancy assists in confirming the diagnosis of secondary lymphedema.

  
  
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  Congenital lymphedema is evident at birth or within the first 2 years of life; lymphedema praecox most commonly presents at puberty and lymphedema tarda after age 35.^1,4

  
  
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  Family history of lymphedema may be present.

Physical Examination

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  Most of the time the diagnosis is clinical, with no need for confirmatory imaging tests.²

  
  
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  Edema may be pitting at early stages, but is nonpitting as the disease becomes chronic.^2,4

  
  
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  Squared-off toes are characteristic of primary lymphedema; the toes have a blunt, squared appearance (Figure 66-1).^1,2,4,5

  
  
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  Dorsal hump may occur, which presents as noticeable edema involving the dorsum of the foot (Figure 66-2).

  
  
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  Stemmer sign is the inability to tent the skin at the base of the second toe.^{1,2,4,5, and 6}

  
  
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  Peau d’orange is a late-stage sign (Figure 66-3); dilated hair follicles with minimal pitting in the skin, cutaneous fibrosis.^2,5

  
  
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  Other skin involvement: hyperkeratosis (Figure 66-4), papillomatosis (Figures 66-5 and 66-6).⁴

  
  
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  In infectious cellulitis (Figure 66-7), the skin appears erythematous, with increased temperature and lymphangitic streaks toward the lymph nodes.⁴

  
  
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  Assess the area in between toes for tinea pedis, as it is a risk factor for cellulitis.⁴

  
  
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  Surgical scars, masses, and other skin lesions can help with the diagnosis of secondary lymphedema.