Polycythemia vera presenting as complex regional pain syndrome of the lower limbs

A 73-year-old woman with bilateral severe pain in her legs and feet was admitted to the hospital. The pain had started spontaneously without any known trauma a year earlier and had progressively worsened to the point that she was unable to walk. The patient complained of pain, discoloration, and sweating of the lower extremities. Physical examination revealed swollen, cold, wet, and blue extremities, with both allodynia and hyperalgesia, fulfilling criteria for a diagnosis of complex regional pain syndrome (CRPS) type 1. Laboratory findings demonstrated a hemoglobin level of 180 g/L(-1) and a hematocrit of 47%, leading to a diagnosis of polycythemia vera (PCV). Her condition slowly improved after repeated venesection, mild analgesics, and amitriptyline.

Polycythemia vera has not been described previously as a contributing factor in CRPS. In the present case, the diagnosis of PCV was important, because its treatment also improved the CRPS.