 |
|
Natural History of Erythromelalgia
Presentation and Outcome in 168 Patients
Mark D. P. Davis, MB, MRCPI;
W. Michael O'Fallon, PhD;
Roy S. Rogers III, MD;
Thom W. Rooke, MD
Arch Dermatol. 2000;136:330-336.
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.
From the Department of Dermatology (Drs Davis and Rogers), Section of Biostatistics (Dr O'Fallon), and Section of Vascular Medicine (Dr Rooke), Mayo Clinic and Mayo Foundation, Rochester, Minn.
RELATED ARTICLES
Erythromelalgia—A Mysterious Condition?
Cato Mørk and Knut Kvernebo
Arch Dermatol. 2000;136(3):406-409.
EXTRACT
| FULL TEXT
Archives of Dermatology Reader's Choice: Continuing Medical Education
Arch Dermatol. 2000;136(3):438-439.
FULL TEXT
THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES
|
Thermoregulatory Sweat Testing in Patients With Erythromelalgia
Davis et al.
Arch Dermatol 2006;142:1583-1588.
ABSTRACT
| FULL TEXT
Combination gel of 1% amitriptyline and 0.5% ketamine to treat refractory erythromelalgia pain: a new treatment option?
Sandroni and Davis
Arch Dermatol 2006;142:283-286.
FULL TEXT
Lidocaine Patch for Pain of Erythromelalgia: Follow-up of 34 Patients
Davis and Sandroni
Arch Dermatol 2005;141:1320-1321.
FULL TEXT
Primary Erythromelalgia in a Child Responding to Intravenous Lidocaine and Oral Mexiletine Treatment
Nathan et al.
Pediatrics 2005;115:e504-e507.
ABSTRACT
| FULL TEXT
Narrative Review: Diseases That Masquerade as Infectious Cellulitis
Falagas and Vergidis
ANN INTERN MED 2005;142:47-55.
ABSTRACT
| FULL TEXT
Alleviation of Erythromelalgia With Venlafaxine
DiCaudo and Kelley
Arch Dermatol 2004;140:621-623.
FULL TEXT
Mutations in SCN9A, encoding a sodium channel alpha subunit, in patients with primary erythermalgia
Yang et al.
J. Med. Genet. 2004;41:171-174.
ABSTRACT
| FULL TEXT
Erythromelalgia: Vasculopathy, Neuropathy, or Both?: A Prospective Study of Vascular and Neurophysiologic Studies in Erythromelalgia
Davis et al.
Arch Dermatol 2003;139:1337-1343.
ABSTRACT
| FULL TEXT
Pathological C-fibres in patients with a chronic painful condition
Orstavik et al.
Brain 2003;126:567-578.
ABSTRACT
| FULL TEXT
The use of regional anaesthetic blockade in a child with recurrent erythromelalgia
Harrison et al.
Arch. Dis. Child. 2003;88:65-66.
ABSTRACT
| FULL TEXT
Lidocaine Patch for Pain of Erythromelalgia
Davis and Sandroni
Arch Dermatol 2002;138:17-19.
FULL TEXT
A Better Understanding of Erythromelalgia
Journal Watch Dermatology 2000;2000:2-2.
FULL TEXT
Erythromelalgia--A Mysterious Condition?
Mork and Kvernebo
Arch Dermatol 2000;136:406-409.
FULL TEXT
|
