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The lymph ends its journey in the thoracic duct or right lymphatic duct, which drain into the blood circulation. [10] Several research groups have hypothesized that chronic inflammation is a key regulator in the development of lymphedema. Th cells, particularly Th2 differentiation, play a crucial role in the pathophysiology of lymphedema.
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.
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 ...
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 ...
Hennekam syndrome, also known as intestinal lymphagiectasia–lymphedema–mental retardation syndrome, [1] is an autosomal recessive disorder consisting of intestinal lymphangiectasia, facial anomalies, peripheral lymphedema, and mild to moderate levels of growth and intellectual disability.
Podoconiosis causes bilateral yet asymmetrical leg swelling with overlying firm nodules. Early on, symptoms may include itching, tingling, widening of the forefoot, and swelling which then progress to soft edema, skin fibrosis, papillomatosis, and nodule formation resembling moss, giving rise to the disease's alternate name of "mossy foot" in some regions of the world. [3]
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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.
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