From the Guidelines
Erythromelalgia is a rare disorder characterized by episodic, burning pain associated with erythema and warmth of the extremities, particularly the feet and hands, which can significantly impact quality of life, increase morbidity and mortality, and is essential to diagnose and manage effectively to avoid severe complications 1.
Definition and Characteristics
Erythromelalgia (EM) is a rare neurovascular disorder that causes episodes of burning pain, redness, and increased skin temperature primarily affecting the extremities. It occurs when blood vessels periodically dilate, causing increased blood flow and inflammation in the affected areas. Episodes are typically triggered by heat exposure, exercise, or pressure on the affected limbs, and can be relieved by cooling the area.
Diagnosis and Classification
Erythromelalgia can be primary (genetic or idiopathic) or secondary to underlying conditions like blood disorders, autoimmune diseases, or medication side effects. The terms primary EM and secondary EM have been advocated, but the use of these terms varies and can be confusing. Conventionally, secondary EM is used for EM with an identifiable underlying cause; primary EM is used for EM with no identifiable underlying cause (idiopathic EM) 1.
Treatment and Management
Treatment of erythromelalgia focuses on avoiding triggers, lifestyle modifications, and pharmacologic treatments, including topical and systemic medications, with a step-wise approach starting with first-line therapy, followed by second-line and third-line therapy if necessary 1.
- First-line therapy includes topical therapy and aspirin 325 mg orally daily for a trial of 1 month.
- Second-line therapy includes systemic therapy, such as gabapentin, pregabalin, or amitriptyline, for a trial of 3-4 months.
- Third-line therapy includes referral to a pain rehabilitation center. Patients should avoid heat exposure, wear loose-fitting clothes and shoes, and elevate affected limbs during episodes. Cooling the affected areas safely, such as ice and water immersion of extremities limited to 10 minutes at a time and 4 times daily, can also help alleviate symptoms. Healthcare may temporarily help alleviate symptoms during EM flares 1.
From the Research
Definition and Characteristics of Erythromelalgia
- Erythromelalgia is a rare clinical condition characterized by intense burning pain, pronounced erythema, and increased skin temperature 2, 3, 4, 5, 6
- It can be divided into primary and secondary forms, with primary erythromelalgia beginning spontaneously at any age and secondary erythromelalgia associated with various medical conditions 2, 3, 5
Symptoms and Diagnosis
- Symptoms of erythromelalgia include severe pain, redness, and hotness in extremities, often triggered by exposure to warmth, pressure, or exercise 2, 3, 5
- Diagnosis is based on medical history and clinical findings, with no diagnostic tests available 2, 3
Treatment and Management
- Various medications, including aspirin, propranolol, and anticonvulsants, have been used to treat erythromelalgia, with some patients experiencing relief 2, 5
- Procedural interventions, such as epidural infusion and sympathetic ganglion block, have also been explored as treatment options, although evidence is limited 4
Associated Conditions and Complications
- Erythromelalgia has been linked to various myeloproliferative disorders, including polycythemia vera and essential thrombocythemia 5
- Symptoms of erythromelalgia can precede the onset of a myeloproliferative disease, highlighting the importance of monitoring patients with erythromelalgia for underlying conditions 5