A 17-year-old girl presents with slowly progressive swelling of her left leg that began around the ankle and dorsum of the foot at puberty. The swelling was initially soft and pitting, but is now firm, brawny and non-pitting. Skin at the base of the second toe cannot be pinched and lifted. Which is the most likely diagnosis?

• A Congenital lymphoedema (Milroy disease)
• B Lymphoedema praecox ✓
• C Lymphoedema tarda
• D Chronic venous insufficiency with lipodermatosclerosis

Explanation

Primary lymphoedema is classified by age of onset: congenital before two years (Milroy disease), praecox between puberty and about 25 years, and tarda after 35 years. Onset at puberty in a teenage girl with distal-to-proximal progression fits lymphoedema praecox, the commonest primary form and predominantly affecting females. A positive Stemmer sign, inability to pinch the skin at the second toe base, confirms lymphoedema and separates it from venous oedema, which spares the dorsum of the foot early.

Reference: Bailey and Love's Short Practice of Surgery, 28th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.