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Lymphedema, also known as lymphoedema and lymphatic edema, is a condition of localized swelling caused by a compromised lymphatic system. [2] The lymphatic system functions as a critical portion of the body's immune system and returns interstitial fluid to the bloodstream.
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.
Secondary lymphedema is a condition characterized by swelling of the soft tissues in which an excessive amount of lymph has accumulated, and is caused by certain malignant diseases such as Hodgkin's disease and Kaposi sarcoma. [1]: 849
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.
Milroy's disease is also known as primary or hereditary lymphedema type 1A or early onset lymphedema. It is a very rare disease with only about 200 cases reported in the medical literature. Milroy's disease is an autosomal dominant condition caused by a mutation in the FLT4 gene which encodes the vascular endothelial growth factor receptor 3 ...
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 ...
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.
Aagenæs syndrome, Cholestasis-lymphedema syndrome Aagenaes syndrome is inherited in an autosomal recessive manner Aagenaes syndrome is a syndrome characterised by congenital hypoplasia of lymph vessels , which causes lymphedema of the legs and recurrent cholestasis in infancy, and slow progress to hepatic cirrhosis and giant cell hepatitis ...
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