ABSTRACT
This chapter includes recommendations from the International Society of Lymphology, National Lymphoedema Network and Union Internationale de Phlebologie. Lymphoedema is chronic progressive swelling in a region due to excessive build-up of protein-rich interstitial fluid. It develops when the lymphatic load is consistently greater than the lymphatic transport capacity in a specific lymphatic territory. Primary lymphoedema has various manifestations. It is a truncular lymphatic malformation due to a developmental abnormality causing hypoplasia or aplasia of lymphatic vessels, lymphatic valvular incompetence or functional defects. The most common complication is infection in stagnant lymph causing cellulitis or lymphangitis which causes further damage to compromised lymphatic vessels and regional lymph nodes. Cellulite is a condition of unknown aetiology in which underlying fat deposits give the skin a dimpled lumpy appearance. Myxoedema from hypothyroidism has a similar appearance to lymphoedema, but the skin is more dry, rough, cool and pale, with coarse hair and hair loss.
