Lymphedema

Background

• Lymphedema is due to abnormal accumulation of interstitial fluid and fibroadipose tissue.
• Occurs with lymphatic load exceeds capacity in the lymphatic system

Etiology

Primary

• Congenital Lymphedema (6-12%) - before age 2
• Lymphedema Precox (77-94%) - at onset of puberty
• Lymphedema Tarda (11%) - after age 35

Secondary

• Cancer and cancer treatment (Lymphadenectomy, Radiotherapy)
  • Breast Cancer - most common
  • Sarcoma
  • Lower Extremity Melanoma
  • Gynecologic Cancer
  • Genitourinary Cancer
  • Head and neck cancer
• Infection
  • Lymphatic Filariasis
  • Tuberculosis
  • Recurrent Skin Infections (eg. Cellulitis, Erysipelas)
  • Lymphadenitis
• Obesity
• Inflammatory disorders
  • Dermatitis
  • Sarcoidosis
  • Arthritis
    • Rheumatoid Arthritis
    • Psoriatic Arthritis
    • Juvenile Idiopathic Arthritis
  • Dermatitis

Clinical Features

• Slowly progressive swelling
• Feeling of heaviness, tightness, or discomfort
• May be pitting at onset
• Dermal thickening with skin becoming dry, firm, and hyperkeratotic
  • Occurs due to cutaneous fibrosis and adipose deposition.
• Stemmer Sign - positive if unable to pinch and lift skin at the base of second toe or finger.

Differential Diagnosis

• Venous Insufficiency
• Acute deep vein thrombosis
• Post-thrombotic Syndrome
• Limb Hypertrophy
  • Hypertrophy of soft tissue or bone (Klippel-Trenaunay syndrome)
  • Overgrowth of body part (Proteus Syndrome)
• Myxedema
• Lipedema
• Tumor