Primary upper-limb lymphoedema.

Background: Lymphoedema is a general term used to designate pathological, regional accumulation of protein-rich fluid. It can be either primary or secondary, and mainly occurs after cancer treatment.

Objectives: To analyse the clinical and lymphoscintigraphic characteristics of primary upper-limb lymphoedema (ULL).

Methods: All of the patients with ULL were recruited at a single Department of Lymphology between January 2007 and December 2011.

Results: In total, 60 patients (33 female, 27 male) were enrolled. For the 54 noncongenital lymphoedemas, the mean age at onset was 38·5 (range 3-82) years. Lymphoedema was unilateral in 51 patients (85%). It always affected the hand, and less often the forearm (55%) or upper arm (23%). Eleven patients (18%) developed cellulitis after onset of lymphoedema, and 21 patients (35%) had associated lower-limb lymphoedema (LLL). Forty-six patients (with 49 lymphoedematous limbs) underwent lymphoscintigraphy: axillary lymph node uptake was diminished in 18 (37%), absent in 24 (49%) and normal in seven limbs (14%). Among the 43 patients with unilateral lymphoedema and lymphoscintigraphy, 28 had epitrochlear node visualization, suggesting a rerouting through the deep lymphatic system, with 15 only on the lymphoedematous limb and 22 on the contralateral nonlymphoedematous limb. The median follow-up period was 103 months, and 57/60 patients (95%) considered their lymphoedema to be stable.

Conclusions: Primary ULL appears later in life than LLL, without predominance in either sex. Infectious complications are rare and patients considered the lymphoedema volume stable throughout life.