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The primary lymphedema is a pathological interstitial accumulation of lymphatic fluid. Lower limbs are mainly affected by primary lymphedema. It is caused by a hereditary lack or complete absence of lymphatic vessel systeme. Final complications of chronic limb lymphedema include »elephantiasis«, lymphatic ulceration and malignant degeneration. Secondary lymphedema, obesity, phlebedema and lipedema are most important differential diagnoses.Treatment of primary lymphedema includes early manual lymph drainages, decrease in weight, exercise, prevention or therapy of skin infections. Up to the present primary lymhedema cannot be cured in causality.