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An apparently healthy 13 year old girl noted progressive swelling of the left leg as her shoe was becoming increasingly tight. A general examination was unremarkable apart from non‐pitting oedema of the left leg without any signs of inflammation. Further questioning revealed that her mother also had a similar “shoe problem” and was thoroughly investigated in 1980s without any specific diagnosis (fig 11).). Her maternal grandmother who lives abroad was reported to be on diuretics for leg swelling.
Primary lymphoedema (praecox subtype) was confirmed by lymphoscintigraphy which showed markedly reduced lymphatic drainage from the left leg (fig 22).
Lymphoedema praecox is the most common type of primary lymphoedema and usually affects females. Although by definition it can present up to 30 years of age, it mostly manifests during puberty. About 70% cases are unilateral, with left lower extremity predominance.1 In these patients lymphatic transport capacity is reduced due to hypoplastic lymphatic channels. This can be confirmed by lymphoscintigraphy which has become the investigation of choice.2
Management is usually conservative with weight loss, good hygiene and avoidance of local trauma/tight garments being most useful. Diuretics have not been found to be of much benefit. In severe cases surgery may be helpful.
Competing interests: None.
Parental/guardian informed consent was obtained for publication of the person's details in this report.