What is lymphedema?

Lymphedema is a chronic medical condition where protein-rich fluid abnormally collects in the interstitium of any region of the body resulting in an upregulated inflammatory response and increased fibrosis that predisposes the afflicted individual to repeat infections and hindered function.

Lymphedema is classified as either primary or secondary. Primary lymphedemas reflect inborn or genetic disorders of the lymphatic system. Affected individuals present with a variety of abnormalities of lymphatic channels and/or lymph nodes, either i.) aplasia (absence of critical structures as in Milroy's Disease); ii.) hypoplasia (normal anatomy but reduced numbers of critical structures); or iii.) dysplasia (abnormal anatomy or function of critical structures). Interestingly, not all primary lymphedemas present at birth, but become evident in teenage years (lymphedema praecox) or later typically after the age of 35 (lymphedema tarda). Later presentations may or may not be associated with ipsilateral soft tissue trauma as the frequently reported "cheerleader syndrome". Further, there are a variety of congenital syndromes associated with lymphedema (i.e. Klippel-Trenaunay Syndrome, Turner's Syndrome, and Noonan's Syndrome) that may complicate diagnosis and treatment as lymphedema in these disorders may be mixed in nature [read more].