How is lymphedema classified?

Updated: Mar 24, 2021
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: William D James, MD  more...
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Answer

Lymphedema is classified into primary and secondary forms. Primary lymphedema (which results from genetic factors) is caused by abnormalities in the lymphatic system that are present at birth, although not always clinically evident until later in life. Primary lymphedema can also be associated with various cutaneous syndromes. The three categories of primary lymphedema are as follows (see Etiology and Workup):

  • Congenital lymphedema (Milroy disease)

  • Lymphedema praecox (Meige disease)

  • Lymphedema tarda

Secondary lymphedema occurs as a result of obstruction of lymphatic flow by known mechanisms, including the following (see Etiology and Workup):

  • Filariasis

  • Silica

  • Podoconiosis, which occurs in barefoot subsistence farmers after walking on red volcanic soil, mostly commonly in Ethiopia [4]

  • Obstruction by a proximal mass

  • Postsurgical mechanisms - Eg, mastectomy

  • Fibrosis secondary to chronic infections

  • Unilateral lower extremity edema (as a complication of non-Hodgkin lymphoma) [5]

  • Massive obesity (termed massive localized lymphedema or obesity-related lymphedema) [6, 7] : The risk of lower extremity lymphedema has been correlated with body mass index (BMI), such that BMI less than 40 is 0%, BMI of 40-49 is 17%, BMI of 50-59 is 63%, BMI of 60-69 is 86%, BMI of 70-79 is 91%, and BMI of 80 or greater is 100%. [8]

  • Edema (giant penile elephantiasis) after circumcision of the penis [9]


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