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Lymphoedema

Lymphoedema is chronic swelling from impaired lymphatic drainage — primary (congenital or appearing at puberty) or secondary (usually after cancer treatment, and worldwide mainly from parasitic infection); a positive Stemmer sign supports the diagnosis, imaging is lymphoscintigraphy, and basic treatment is complete decongestive therapy (manual drainage, compression bandaging, exercise and skin care) followed by lifelong compression garments.

Why it matters in the exam

In primary lymphoedema there is a congenital form (Milroy, linked to VEGFR3), a form appearing around puberty (praecox — the commonest primary form, mainly in women) and a late form after age 35 (tarda). In secondary lymphoedema the commonest cause in developed countries is cancer treatment — axillary or groin node dissection and radiotherapy (for example after breast cancer) — and worldwide filariasis (Wuchereria bancrofti). The swelling starts as soft pitting oedema, and later becomes hard, fibrotic and non-pitting, with skin thickening. Stemmer sign — inability to pinch the skin at the base of the second toe — is positive in lymphoedema. The differential diagnosis includes venous oedema, heart failure, hypoalbuminaemia, DVT and lipoedema — symmetrical fat deposition in the legs of women, which is painful and spares the feet.

ISL stages: 0 — latent, no visible swelling; I — soft oedema that subsides with limb elevation; II — oedema that does not subside with elevation, with early fibrosis; III — severe skin thickening ("elephantiasis") with skin changes. The core treatment is conservative: in the intensive phase — manual lymphatic drainage, multilayer compression bandaging, exercise and skin care; in the maintenance phase — fitted compression garments for life. Diuretics are not effective in lymphoedema. Recurrent skin infections (cellulitis) are common and worsen the swelling, and in a patient with repeated episodes prophylactic penicillin is given. Surgical options: physiological operations — lymphaticovenous anastomosis (LVA) or vascularised lymph node transfer — in early stages, and liposuction or excision (Charles) in late stages with fatty and fibrotic tissue. A rare and serious complication is lymphangiosarcoma (Stewart–Treves) in a limb with long-standing oedema, for example years after breast cancer treatment.

In new arm swelling after cancer treatment, tumour recurrence in the axilla or venous thrombosis must be excluded before attributing it to the lymphatics alone.

Key points

Summary table

ISL stageFindingTreatment
0No visible swelling, impaired transportFollow-up and education
ISoft oedema that subsides with elevationDecongestive therapy and compression maintenance
IIDoes not subside with elevation, early fibrosisCombined therapy; LVA or node transfer in selected patients
IIIThick, hard skin, skin changesCompression, skin care; liposuction or excision

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Sources

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Reviewed by the Examqo AI Professor · 2026-11-06. The text may contain errors; when in doubt, check the source.