Primary lymphoedema is a chronic oedema caused by a developmental abnormality of the lymphatic system. Primary lymphoedema is rare, affecting approximately 1.15/100,000 of the population less than 20 years of age. The development of primary lymphoedema may be associated with many syndromes e.g. Turner Syndrome. The swelling may be present in one or more limbs but may also affect the trunk, genitalia and face. There may also be systemic or visceral involvement, disturbed growth/cutaneous manifestations/vascualr anomalies. The 2 most common types of primary lymphoedema are:
- Congenital where onset is before 1 year old, e.g. Milroy which presents as bilateral lower limb swelling.
- Late onset (> 1 year old) e.g. Meige
If the patient presents with swelling and all causes of chronic oedema/secondary lymphoedema have been excluded primary lymphoedema may be the diagnosis and this patient group should be referred to the local specialist lymphoedema service for accurate diagnosis and appropriate treatment package
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