Summary

• Chronic, progressive swelling of tissue with protein-rich fluid as a consequence of developmental (primary lymphoedema) or acquired (secondary lymphoedema) disruption of the lymphatic system. Extremities are most commonly affected, followed by genitalia.
• Most cases are secondary to nematode infection (filariasis), malignancy, or cancer-related treatment.
• Typically presents with painless unilateral limb swelling; pitting oedema is present in early disease, whereas non-pitting oedema is a sensitive but non-specific finding in advanced disease.
• Diagnosis is usually made on clinical grounds and confirmed by lymphoscintigraphy.
• First-line treatment involves compression, ranging from static garments to complex massage and pneumatic compression devices. Surgical procedures are reserved for patients refractory to conservative measures and/or with significant morbidity.
• There is no cure. Successful care requires a long-term, collaborative approach between patient and providers.