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Erythromelalgia: Identification of a corticosteroid-responsive subset.
Gabriel L Pagani-Estévez1, Paola Sandroni1, Mark D Davis2
1Department of Neurology, Mayo Clinic, Rochester, Minnesota.
Journal of the American Academy of Dermatology
|April 18, 2017
Summary
Corticosteroid treatment for erythromelalgia may be more effective when initiated sooner and following a precipitating event. Early intervention might be key before irreversible nerve changes occur.
Area of Science:
- Neurology
- Dermatology
- Pharmacology
Background:
- Erythromelalgia is a rare condition characterized by intense burning pain, redness, and heat, typically affecting the extremities.
- Corticosteroids (CS) are sometimes used to manage erythromelalgia symptoms, but predictors of response are not well-defined.
Purpose of the Study:
- To identify clinical predictors associated with corticosteroid responsiveness in patients diagnosed with erythromelalgia.
Main Methods:
- Retrospective case series analysis of 31 erythromelalgia patients who received corticosteroid treatment.
- Patients were categorized into non-responders (NRs), partial responders (PSRs), and complete responders (CSRs).
- Statistical analysis (P < .05) was used to determine significant predictors.
Main Results:
- A subacute onset (less than 21 days to peak symptoms) was associated with a higher likelihood of being a steroid responder (SR) (87%, P = .003).
- A disease precipitant (infection, trauma, surgery) was reported by 67% of complete responders (P = .007).
- Steroid responders received CS treatment significantly sooner (3 months) compared to non-responders (24 months) (P = .003).
Conclusions:
- Subacute presentation and preceding infectious, traumatic, or surgical events may indicate a better response to corticosteroids in erythromelalgia.
- An early therapeutic window for CS intervention may exist, potentially before nociceptive remodeling and central sensitization become significant.