A 44-year-old Caucasian woman with type I diabetes presented with massive localized lymphedema (MLL), a phenotype of lymphedema. She had been overweight most of her adult life, with most of her body weight being disproportionally distributed in her lower body. However, actual leg swelling had begun about 10 years ago. She was either scratched or bitten on the left calf and developed cellulitis with sepsis. The left leg edema continued to worsen even after the cellulitis resolved until it was profoundly enlarged, and gradually the right leg began to enlarge as well. The pendulous enlargements at the back of both thighs began slowly and continued to increase over the years until they began to affect her ability to walk. She had undergone a lap band for morbid obesity 2 years ago and had lost more than 45 kg, but this had little effect on her leg enlargement. Compression stockings would not fit.

On clinical examination she was found to have stage III lymphedema with elephantiasis and massive localized lymphedema on both the legs, the left being worse than the right (Figures 71-1 and 71-2). Her right foot was spared, but the left foot exhibited a positive Stemmer sign (the inability to pinch a fold of skin at the base of the second toe), which is diagnostic of lymphedema. The patient responded very well to standard treatment for lymphedema with complete decongestive physiotherapy (Figures 71-3 and 71-4). She was provided with custom garments and a pneumatic compression device to help her with long-term management.

It is common for patients to relate the onset of lymphedema to an episode of cellulitis. However, it can be difficult to determine whether the cellulitis was due to her lymphedema, or the other way around. Her age of onset, the fact that her foot was affected, and the severity of its progression are factors that suggest she might originally have had a form of primary lymphedema (lymphedema tarda), which presents about the age of 35. In other words, inherently inadequate lymphatic function may have predisposed her to cellulitis (a problem for which she was even at higher risk due to diabetes mellitus). The trigger for the development of clinically apparent lymphedema was likely the episode of cellulitis, with subsequent damage to the lymphatics after the cellulitis worsened lymphatic transport. Under these conditions a vicious cycle can develop, and this may result in recurrent episodes of cellulitis with continued worsening of lymphatic function and progressive edema. The patient also may have a genetic fatty deposition disorder called lipedema, which causes fat to be preferentially deposited below the waist. In some cases, patients with this type of leg enlargement progress to lymphedema. Most patients with lipedema have sparing of their feet until quite late. In patients like this, it can be challenging to determine whether they have primary (congenital) or secondary lymphedema, and these definitions may become obsolete, as better lymphatic imaging now suggests lymphatic transport capacity ranges along a continuum. Treatment differs little regardless of etiology.

Lipedema

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  Genetic disorder of fatty deposition, usually below the waist

  
  
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  Usually presents in adulthood (typically 30 years onward)

  
  
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  Progression is typically slow (period of years)

  
  
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  Almost exclusively affects females so there is an assumed hormonal influence¹

Lipolymphedema

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  Some patients with lipedema progress to develop lymphedema (thus, lipolymphedema), when swelling is persistent and the feet become affected (Figure 71-5). Cutaneous fibrosis suggests superimposed lymphedema has evolved as well.

  
  
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  Rare, but may be increasing due to marked increase in obesity levels in Western populations.

MLL

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  Characterized by extreme localized enlargement of the limb (usually the thigh but can be the upper arm) due to lymphatic fluid and its associated inflammatory tissue changes.

  
  
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  MLL is more common in morbidly obese patients.²

  
  
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  Although lipolymphedema is rare in males, a substantial proportion of MLL patients are males.^2,3

  
  
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  Previously described in the literature as a rare disorder, in a clinic-based population of patients with lower extremity lymphedema, 1.3% had MLL.⁴

Lipolymphedema

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  Most common etiology appears to be initial lipedema with progression to secondary lymphedema, which can take as long as 17 years according to one study.^5,6 In this case, the lipedema—deposition of fat around the buttocks and legs but sparing of the feet (pantaloon appearance)—becomes more widespread as the lymphedema progresses. Although the mechanism for development of lipolymphedema is not fully understood, it is believed that in some instances the fat deposition from lipedema causes damage to lymphatic and capillary systems. A subclinical phenotype of primary lymphedema may also be present that predisposes the lower extremities toward lymphedema particularly after trauma or injury.¹

MLL