Lymphoedema is classified into different types based on the origin or cause of the lymphoedema. Primary or Secondary Lymphoedema.
PRIMARY LYMPHOEDEMA
CONGENITAL LYMPHOEDEMA
- Presents in very young children at birth to 2 years
- Accounts for 5-10% of all primary lymphoedemas
- Normally affects the legs, but arms, face and genitalia may also be affected
- Affects females more than males
LYMPHOEDEMA PRAECOX
- Develops after puberty, often within the 2nd and 3rd decades of life, before 35years of age
- Accounts for 80% of all primary lymphoedemas
- Affects mainly females
LYMPHOEDEMA TARDA
- Starts after the age of 35 years
- Accounts for 10% of all primary lymphoedemas
- Affects mainly females
This is as a result of a developmental deficiency in the anatomy of the lymphatic system i.e. the lymphatic vessel or lymph nodes have no developed completely before birth. This may be due to a familial predisposition or a congenital cause. There are different types:
- Aplasia (absence of lymphatic vessels)
- Hypoplasia (Decreased number or unusually small lymphatics)
- Hyperplasia (Increased in size and number but with valves that malfunction)
- Obstruction of lymphatic vessels
SUBDIVISIONS OF HEREDITARY LYMPHOEDEMA
- Congenital hereditary lymphoedema
- Hereditary lymphoedema, type I
- Lymphoedema-distichiasis
- Lymphoedema praecox
- Lymphoedema tarda
- Milroy disease
- Nonne-Milroy disease
- Turner Syndrome, Klinefelter Syndrome, Noonan Syndrome, yellow nail syndrome or Kinmonth Syndrome (fibrosis of the lymph nodes), Klippel-Trenaunay-Weber Syndrome
Primary lymphoedema mostly affects the lower limb (unilateral or bilateral) but can affect the arms and other areas. In the leg, the swelling usually starts in the foot and gradually moves up the limb. There may or may not have been a trigger event such as minor trauma, infections or immobility. Often the swelling is mild at first but progresses without treatment.
Hereditary lymphoedema is a genetic developmental disorder affecting the lymphatic system. It is characterized by swelling (oedema) of certain parts of the body. The lymphatic system is a circulatory network of vessels, ducts, and nodes that filter and distribute certain protein-rich fluid (lymph) and blood cells throughout the body. In hereditary lymphedema, lymphatic fluid collects in the subcutaneous tissues under the epidermis due to obstruction, malformation, or underdevelopment (hypoplasia) of various lymphatic vessels. There are three forms of hereditary lymphedema: congenital hereditary lymphedema or Milroy disease; lymphoedema praecox or Meige disease; and lymphoedema tarda.
Symptoms include swelling (lymphoedema) and thickening and hardening of the skin in affected areas. In most cases, hereditary lymphoedema is inherited as an autosomal dominant trait.
Lipoedema is a symmetrical accumulation of abnormal collection of subcutaneous fat, most often in the legs. Lipoedema occurs almost exclusively in females 85% and 15 % male. Tenderness and bruising is common. Typically, the feet are not swollen and no thickening of the skin of the toes or dorsum of the feet (no Stemmer’s sign). Lipoedema is frequently misdiagnosed as lymphoedema.
