What is the prognosis of erythromelalgia?

Updated: Sep 17, 2018
  • Author: Antoine N Saliba, MD; Chief Editor: Emmanuel C Besa, MD  more...
  • Print

A retrospective review of 168 patients with erythromelalgia examined at the Mayo Clinic between 1970 and 1994 showed a significant decrease in survival compared with that expected for age- and sex-matched control subjects. [17] However, a recent Swedish study that collected data retrospectively for a period of 10.5 years reported no mortality directly related to erythromelalgia, although three patients developed intra-abdominal malignancies on follow-up. [25]

Patients who respond to aspirin have little morbidity. Spontaneous remissions may occur. Early-onset disease is relatively unresponsive to treatment and generally is unremitting. Complications appear to be more frequent in patients who clearly have platelet-mediated disease.

Ulceration, necrosis, and gangrene of affected extremities are possible. Digital necrosis or skin ulceration with secondary infection can lead to amputation. At least one patient had near-fatal hypothermia related to constant cooling required to control symptoms.

Did this answer your question?
Additional feedback? (Optional)
Thank you for your feedback!