Natural history of erythromelalgia: presentation and outcome in 168 patients.
OBJECTIVE
To describe the demographics, presentation, and outcome in patients with erythromelalgia--a rare and poorly understood clinical syndrome defined by the triad of red, hot, painful extremities.


METHODS
Retrospective medical record review with follow-up by survey questionnaire.


METHODS
Large tertiary care medical center.


METHODS
Patients with erythromelalgia examined at the Mayo Clinic, Rochester, Minn, between 1970 and 1994.


METHODS
The medical records of 168 patients were analyzed. Follow-up data, which consisted of answers to 2 survey questionnaires or the most recent information in the medical record from patients still alive and death certificates or reports of death for those deceased patients, were obtained for all but 13 patients.


METHODS
Survival, morbidity, and quality of life.


RESULTS
All patients were white; 122 (72.6%) were female, and 46 (27.4%) were male. At presentation, the patients' mean age was 55.8 years (age range, 5-91 years). Symptoms had been present since childhood in 7 patients (4.2%). Six patients (3.6%) had a first-degree relative with erythromelalgia. Symptoms were intermittent in 163 patients (97.0%) and constant in 5 (3.0%). Symptoms predominantly involved feet (148 patients [88.1%]) and hands (43 patients [25.6%]). Kaplan-Meier survival curves revealed a significant decrease in survival compared with that expected in persons of similar age and of the same sex (P<.001). After a mean follow-up of 8.7 years (range, 1.3-20 years), 30 patients (31.9%) reported worsening of, 25 (26.6%) no change in, 29 (30.9%) improvement in, and 10 (10.6%) complete resolution of the symptoms. On a standard health status questionnaire, scores for all but one of the health domains were significantly diminished in comparison with those in the US general population.


CONCLUSIONS
Erythromelalgia is a syndrome with significantly increased mortality and morbidity compared with the US general population.
Davis
M D
MD
Department of Dermatology, Mayo Clinic and Mayo Foundation, Rochester, Minn 55905, USA.
O'Fallon
W M
WM
Rogers
R S
RS
Rooke
T W
TW
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D002675:Child, Preschool; D004916:Erythromelalgia / Q000453:epidemiology / Q000401:mortality / Q000473:pathology / Q000503:physiopathology*; D005260:Female; D005500:Follow-Up Studies; D006306:Health Surveys; D006801:Humans; D008297:Male; D008499:Medical Records; D008875:Middle Aged; D009017:Morbidity; D011788:Quality of Life; D012189:Retrospective Studies; D011795:Surveys and Questionnaires; D016019:Survival Analysis