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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.
Hereditary lymphedema is a primary lymphedema – swelling that results from abnormalities in the lymphatic system that are present from birth. Swelling may be present in a single limb, several limbs, genitalia, or the face. It is sometimes diagnosed prenatally by a nuchal scan or postnatally by lymphoscintigraphy. [medical citation needed]
Primary lymphedema is sometimes associated with other genetic disorders as well, like Turner syndrome. Diagnosis early on is difficult with lymphedema since there aren’t many obvious signs, and can sometimes mimic chronic venous insufficiency, or CVI, where blood collects in the veins, though usually this happens bilaterally, meaning on both ...
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 ...
Meige disease is a primary lymphedema that is not caused by another condition; secondary lymphedema is a typical consequence of a mastectomy. Another primary lymphedema is Milroy disease in which the lymphedema is present at birth. [3] Lymphedema tarda occurs after the age of 35. [4] Meige disease has its onset around the time of puberty.
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.
Yellow nail syndrome, also known as "primary lymphedema associated with yellow nails and pleural effusion", [1]: 849 is a very rare medical syndrome that includes pleural effusions, lymphedema (due to under development of the lymphatic vessels) and yellow dystrophic nails. [2] Approximately 40% will also have bronchiectasis.
Lymphedema is the condition of swelling of tissue relating to insufficient clearance by the lymphatic system. [34] It can be congenital as a result usually of undeveloped or absent lymph nodes, and is known as primary lymphedema.