Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2000 Mar;136(3):330-6.
doi: 10.1001/archderm.136.3.330.

Natural history of erythromelalgia: presentation and outcome in 168 patients

M D Davis  1 , W M O'Fallon, R S Rogers 3rd, T W Rooke
Affiliations

Natural history of erythromelalgia: presentation and outcome in 168 patients

M D Davis et al. Arch Dermatol. 2000 Mar.

Abstract

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.

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

Setting: Large tertiary care medical center.

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

Intervention: 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.

Main outcome measures: 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.

Conclusion: Erythromelalgia is a syndrome with significantly increased mortality and morbidity compared with the US general population.

PubMed Disclaimer

Comment in

  • Erythromelalgia--a mysterious condition?
    Mørk C, Kvernebo K. Mørk C, et al. Arch Dermatol. 2000 Mar;136(3):406-9. doi: 10.1001/archderm.136.3.406. Arch Dermatol. 2000. PMID: 10724206 Review. No abstract available.

MeSH terms