Epidemiology


Etiology


  • Primary lymphedema (rare): idiopathic, resulting from congenital abnormalities in lymphatic vessels, e.g., in Turner syndrome
  • Secondary lymphedema: physical disruption of lymphatic drainage
    • Lymphadenectomy, radiation
    • Malignant obstruction
    • Chronic inflammation (eg, recurrent cellulitis)
    • Parasitic infection (eg, filariasis)

Pathophysiology


  • Painless unilateral or bilateral swelling of limbs
    • In early disease, the edema is pitting and the skin remains soft. However, over time there is progressive deposition of subcutaneous collagen and adipose tissue, which leads to firm, dry, and thickened skin and nonpitting edema
    • Swelling of toes and dorsal feet with deep flexion creases
    • Lymphedema involves the toes, unlike venous edema, which typically spares the toes.

Clinical features


Diagnostics


Treatment


  • Manual lymphatic drainage: a form of massage that is applied in the direction of the heart to increase the natural drainage of lymph
  • Compression garments, e.g., multilayer compression stockings (applied by a specialist)
    • Should be applied by a trained professional (e.g., a physiotherapist or clinical lymphologist) as they can be harmful or ineffective when applied incorrectly.