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Primary lymphedema is a form of lymphedema which is not directly attributable to another medical condition. It can be divided into three forms, depending upon age of onset: congenital lymphedema, lymphedema praecox, and lymphedema tarda. [1] Congenital lymphedema presents at birth. Lymphedema praecox presents from ages 1 to 35.
Lymphedema is most frequently a complication of cancer treatment or parasitic infections, but it can also be seen in a number of genetic disorders. Tissues with lymphedema are at high risk of infection because the lymphatic system has been compromised. [3] Though incurable and progressive, a number of treatments may improve symptoms. [2]
The pathophysiology is not yet well understood. Leukocytoclastic vasculitis is proposed to be the underlying cause resulting in reactive lymphedema. [2] Prolonged standing with full knee extension and minimal movement for a prolonged period of time is postulated to induce a temporary failure in pumping the venous and lymphatic systems in the calf region leading to acute gravity-dependent ...
Lymphedema praecox [2] is a condition characterized by swelling of the soft tissues in which an excessive amount of lymph has accumulated, and generally develops in females between the ages of nine and twenty-five. This is the most common form of primary lymphedema, accounting for about 80% of the patients.
Factitial lymphedema, also known as hysterical edema, is a skin condition produced by wrapping an elastic bandage, cord, or shirt around an extremity, and/or holding the extremity in a dependent and immobile state.
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Meige lymphedema, Late-onset lymphedema, Lymphedema hereditary type 2, [1] Meige disease , or Meige lymphedema is a genetic disorder in which lymphedema later develops. [ 2 ] Meige disease is a primary lymphedema that is not caused by another condition; secondary lymphedema is a typical consequence of a mastectomy .
Hypotrichosis–lymphedema–telangiectasia syndrome is a congenital syndrome characterized by lymphedema (swelling of tissue due to malformation or malfunction of lymphatics), the presence of telegiectasias (small dilated vessels near the surface of the skin), and hypotrichosis or alopecia (hair loss). Lymphedema usually develops in the lower ...