What is primary lymphedema and how is it classified?

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

Lymphedema arising from a developmental abnormality of the lymphatic system is classified as primary lymphedema. [11] This form of the disease is divided into the following 3 main types, which are distinguished by their age of onset. (Connell et al proposed a classification system of the primary lymphatic dysplasias that is based on phenotype rather than age of onset. [12] ) These types are as follows:

  • Congenital lymphedema (Milroy disease)

  • Lymphedema praecox (Meige disease)

  • Lymphedema tarda

These conditions involve the lower extremities almost exclusively. All are caused by a congenital abnormality in the lymphatic system, although these defects may not always be clinically evident until later in life, when a triggering event or worsening of the condition causes the lymphatic transport capacity to exceed the volume of interstitial fluid formation; in such cases, the patient is unable to maintain normal lymphatic flow.

Primary lymphedema also can be associated with other cutaneous and genetic disorders not among the 3 main, age-based categories.


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