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Correspondence to Professor Uday Shamrao Kumbhar; [email protected]
Background
Lymphoedema is an abnormal collection of interstitial protein-rich fluid. It is caused either due to an embryological maldevelopment (primary lymphoedema) or an acquired lymphatic obstruction (secondary lymphoedema). Primary lymphoedema is rare to occur. Diagnosis is usually by clinical and imaging findings. Treatment of primary lymphoedema depends on the grade of the disease and includes conservative and surgical approaches. We report a rare case of lymphoedema praecox in an adolescent female diagnosed with primary lymphoedema, diagnosed by imaging and managed conservatively.
Case presentation
A 16-year-old woman presented with swelling of the left lower limb, which was insidious onset over the past 6 years. The oedema increases with activity and decreases on rest and limb elevation. She had no pain, fever or lymphadenopathy. There is no family history of similar spontaneous limb oedema. On examination, there was unilateral oedema of the left lower limb extending from the foot up to the left knee (figure 1).There was a difference in the circumference of the limbs, which was 1 cm at the calf level and 1.5 cm in the ankle, and hence it was more prominent at the ankle level. The skin and its appendages were normal without any symptoms or signs of localised infection.
Oedema was pitting in nature without any erythema, warmth, tenderness or ulceration over the skin. Movements of the knee and ankle joints were normal. There was no ipsilateral or contralateral lymphadenopathy. Although her limbs appeared hairy, she had no other features of hirsutism elsewhere on her body. She does not have any signs or symptoms of other endocrine abnormalities that could lead to hirsutism. Examination of the contralateral lower limb and bilateral upper limbs was unremarkable.
Evaluation for filariasis, venous thrombosis, a malignant condition, liver, renal, cardiac or thyroid diseases were negative. Lymphoscintigraphy was done for further evaluation, which revealed no lymph nodes or lymphatic channels in bilateral lower limbs (figures 2 and 3). As it is the initial stage of lymphoedema, she was managed conservatively with compression stockings with regular follow-up.
Outcomes and Follow-up
The patient was followed up for the next 6 months, and there was no increase in the lymphoedema of the left leg.
Discussion
Lymphoedema is divided into primary and secondary. Primary...