Pediatric erythromelalgia from multidisciplinary perspectives: a scoping review

Area of Science:

• Pediatric Pain Management
• Rare Disease Research
• Systematic Literature Review

Background:

• Erythromelalgia is a rare, chronic pain condition affecting extremities.
• Characterized by burning pain, warmth, and redness, exacerbated by heat.
• Limited research exists on pediatric erythromelalgia, hindering standardized care.

Purpose of the Study:

• To map existing literature on childhood-onset erythromelalgia.
• To identify knowledge gaps in pediatric erythromelalgia research.
• To guide future research directions for this rare condition.

Main Methods:

• Conducted a scoping review of 167 studies.
• Included 411 reported cases of childhood-onset erythromelalgia.
• Analyzed variability in clinical presentation, diagnosis, and management.

Main Results:

• Identified significant variability in symptom reporting and diagnostic criteria.
• Observed diverse clinical assessments, investigations, and treatment plans.
• Highlighted factors for early recognition but noted substantial research gaps.

Conclusions:

• Pediatric erythromelalgia literature shows considerable heterogeneity.
• Standardized data collection is crucial for consensus recommendations.
• A multicenter registry is proposed to benefit clinicians and patients.