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PATIENT STORY

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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.

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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.

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FIGURE 71-1

Anterior view of the patient at presentation.

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FIGURE 71-2

Posterior view of the patient at presentation.

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FIGURE 71-3

Patient response after four weeks of intensive complex decongestive treatment.

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FIGURE 71-4

Current view of lower extremities after eight weeks of intensive complex decongestive treatment.

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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 ...

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