Lymphedema and Paget’s disease: beyond the nipple

Lymphedema praecox is a rare lymphatic vascular anomaly diagnosed before the age of 35, presenting with unilateral involvement of the lower extremities. Paget's disease of bone (PDB) is also a rare osteometabolic disorder characterized by an accelerated rate of bone remodeling, and diagnosis of the disease is challenging. Here, we report a 49-year-old woman with primary lymphedema since the age of 33, who presented with pain and swelling in her right leg. Although the volume of the extremity diminished after complete decongestive therapy, the pain continued. Further evaluation with X-ray, bone scan, and biochemical markers supported the diagnosis of PDB. Following the zoledronic acid infusion, the pain score improved. However, there is no published association between these two diseases. Both lymphedema and PDB are associated with vascular disease and have a genetic background. This is the first case reporting the coexistence of lymphedema and PDB in the same extremity.