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Hub overviewCommon complicationICD-10 I89.0 / Q82.0

Lymphoedema (general overview)

Primary lymphoedema · secondary lymphoedema · lymphatic insufficiency

Lymphoedema is a chronic limb / regional swelling caused by impaired lymphatic drainage. It is classified as primary (congenital / genetic — Milroy disease, Meige disease, lymphoedema-distichiasis) or secondary (most commonly after lymphadenectomy, radiotherapy, infection — filariasis — or trauma). In UK skin-oncology practice it predominantly follows melanoma / breast / vulval-cancer lymphadenectomy. Diagnosis is clinical, supplemented by ISL staging, lymphoscintigraphy and ICG lymphography. Modern management is multidisciplinary — complex decongestive therapy (CDT), compression, and supermicrosurgical reconstruction (LVA, VLNT).

CurrentLast reviewed 16 May 2026
Clinical image of Lymphoedema (general overview)
Lymphoedema (general overview). Image sourced from DermNet New Zealand. Used under CC BY-NC-ND 4.0. No endorsement implied.

Classification

  • Primary lymphoedema:
    • Congenital (Milroy disease, FLT4/VEGFR3 mutation) — present at birth.
    • Lymphoedema praecox (Meige) — onset puberty - 35 years.
    • Lymphoedema tarda — onset >35 years.
    • Syndromic: lymphoedema-distichiasis (FOXC2), Turner, Noonan, Hennekam.
  • Secondary lymphoedema:
    • Post-oncology: lymphadenectomy (breast / melanoma / vulval / penile / gynae), radiotherapy.
    • Filarial: Wuchereria bancrofti in endemic countries (commonest globally).
    • Trauma, infection (recurrent cellulitis).
    • Tumour-related lymphatic obstruction (cutaneous metastases, advanced cancer).
    • Chronic venous insufficiency overlap (phlebolymphoedema).
    • Obesity-related lymphoedema.

ISL staging (2020)

  • Stage 0 (latent): subclinical; impaired transport demonstrable on lymphoscintigraphy.
  • Stage I: pitting oedema reversed by elevation.
  • Stage II: progressive fibrosis; reduced pitting; not fully reversible.
  • Stage III: lymphostatic elephantiasis with trophic skin changes, hyperkeratosis, papillomatosis.

Clinical features

  • Painless heavy swelling; usually unilateral.
  • Stemmer sign positive (cannot pinch second toe / finger fold).
  • Skin changes: peau d'orange, hyperkeratosis, lymphorrhoea, papillomatosis.
  • Recurrent cellulitis episodes.
  • Functional limitation, body-image issues.
  • Complications:
    • Recurrent cellulitis (each episode worsens lymphoedema).
    • Cutaneous angiosarcoma (Stewart-Treves syndrome) — rare but important; persistent / chronic lymphoedema risk; bluish nodules / patches; refer urgently for biopsy.
    • Functional impairment, depression.

Workup

  • Limb circumference measurement; perometer / water displacement.
  • Bioimpedance spectroscopy (L-Dex) — early latent disease.
  • Lymphoscintigraphy — gold standard imaging.
  • ICG lymphography — superficial lymphatics; preoperative LVA planning.
  • MR lymphangiography — complex cases.
  • USS Doppler — exclude DVT / venous component.
  • Bloods if filaria endemic country: blood film, microfilarial PCR.
  • Genetic testing for primary lymphoedema (FLT4, FOXC2 — R124 / R142 gene panels).

Management

  • Complex decongestive therapy (CDT) — first-line:
    • Manual lymphatic drainage (MLD).
    • Multilayer short-stretch compression bandaging.
    • Compression garments (class 1-3) fitted by specialist.
    • Skin care: emollients, infection prevention.
    • Decongestive exercise.
  • Pneumatic compression devices — adjunctive.
  • Pharmacology: limited evidence; coumarin / selenium tried historically; not routinely recommended.
  • Surgical (specialist centres):
    • Lymphaticovenular anastomosis (LVA) — early-stage; supermicrosurgical anastomosis of lymphatic to venule.
    • Vascularised lymph-node transfer (VLNT) — flap-based donor lymph-node transfer to affected basin.
    • Suction-assisted lipectomy / debulking — late-stage fibrofatty disease.
  • Antibiotic prophylaxis: phenoxymethylpenicillin 250 mg BD long-term for ≥2 cellulitis episodes / 12 months (PATCH-II).
  • Multidisciplinary: lymphoedema clinic, physiotherapy, plastic surgery, dermatology, vascular, psychology, dietetics.

References

  1. International Society of Lymphology. Diagnosis and treatment of peripheral lymphedema: 2020 consensus document. Lymphology. 2020;53:3-19.
  2. British Lymphology Society. Standards of practice for the management of lymphoedema. Sevenoaks: BLS; 2023.
  3. Schaverien MV et al. Lymphedema: state-of-the-art review. Plast Reconstr Surg. 2017;140:1003e-1017e.
  4. Lymphoedema Support Network (LSN). UK patient resource. London: LSN; 2024.
  5. NICE NG101. Early and locally advanced breast cancer: diagnosis and management. London: NICE; 2018 (last updated 14 April 2025), recommendations 1.14.8-1.14.11.
  6. NICE HTG622. Liposuction for chronic lymphoedema. London: NICE; 2022.
  7. NICE HTG717. Lymphovenous anastomosis during axillary or inguinal node dissection for preventing secondary lymphoedema. London: NICE; 2024.

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