Summary about Disease
Erythromelalgia is a rare neurovascular peripheral pain disorder characterized by intense, burning pain, redness, and increased skin temperature, primarily affecting the extremities, especially the feet and hands. It can be primary (idiopathic) or secondary, associated with underlying medical conditions. Episodes can be triggered by warmth, pressure, exertion, or even no identifiable trigger.
Symptoms
The primary symptoms include:
Intense burning pain, often described as throbbing, stabbing, or electric shock-like.
Visible redness (erythema) of the affected area.
Increased skin temperature; the affected area feels hot to the touch.
Swelling in some cases.
Symptoms are typically episodic and triggered by warmth, exertion, or pressure.
Symptoms are often relieved by cooling the affected area.
Causes
Erythromelalgia can be either primary or secondary:
Primary Erythromelalgia (Idiopathic): The cause is often unknown. Some cases are linked to genetic mutations, particularly in the SCN9A gene, which affects sodium channels in pain-sensing neurons.
Secondary Erythromelalgia: This form is associated with underlying medical conditions such as:
Myeloproliferative disorders (e.g., polycythemia vera, essential thrombocythemia)
Autoimmune diseases (e.g., lupus, rheumatoid arthritis)
Small fiber neuropathy
Diabetes
Multiple sclerosis
Medications (e.g., calcium channel blockers, some antidepressants)
Infections
Medicine Used
There is no single, universally effective treatment for erythromelalgia. Management focuses on symptom relief and addressing any underlying conditions:
Topical treatments: Capsaicin cream, lidocaine cream
Oral medications:
Pain relievers: Aspirin, NSAIDs (e.g., ibuprofen, naproxen), tramadol
Neuropathic pain medications: Gabapentin, pregabalin, amitriptyline, duloxetine
Vasodilators: Misoprostol
Calcium channel blockers
Other treatments:
IV lidocaine or other intravenous therapies (used in severe cases)
Nerve blocks
Spinal cord stimulation
Is Communicable
No, erythromelalgia is not a communicable disease. It is not contagious and cannot be spread from person to person.
Precautions
Precautions focus on managing triggers and protecting the affected extremities:
Avoid exposure to heat: Limit time outdoors in hot weather, avoid hot baths/showers, and keep the environment cool.
Elevate the affected limbs to reduce swelling.
Wear loose-fitting, breathable clothing and footwear.
Avoid strenuous exercise and activities that increase body temperature.
Stay hydrated.
Protect affected areas from injury or pressure.
Manage underlying medical conditions that may contribute to secondary erythromelalgia.
How long does an outbreak last?
The duration of an erythromelalgia episode varies significantly. Episodes can last from minutes to hours, or even days. The frequency and duration of episodes also vary greatly between individuals.
How is it diagnosed?
Diagnosis is based on clinical evaluation and medical history:
Physical examination: Assessment of skin temperature, color changes, and pain response.
Medical history: Review of symptoms, triggers, and any underlying medical conditions.
Provocation tests: Controlled exposure to warmth to observe symptom onset.
Nerve conduction studies: To rule out other causes of neuropathy.
Blood tests: To evaluate for underlying conditions like myeloproliferative disorders or autoimmune diseases.
Skin biopsy: To assess for small fiber neuropathy.
Timeline of Symptoms
The timeline of symptoms typically involves:
Trigger: Exposure to heat, exertion, pressure, or sometimes no identifiable trigger.
Initial sensation: A tingling or burning sensation may begin gradually.
Escalation: The pain intensifies, accompanied by redness and increased skin temperature.
Peak: Symptoms reach their most severe point.
Relief: Cooling the affected area may bring relief, causing the pain and redness to gradually subside.
Important Considerations
Erythromelalgia can significantly impact quality of life due to chronic pain and limitations on activities.
Early diagnosis and management are important to minimize the impact of symptoms.
Finding effective treatments can be challenging, and a multidisciplinary approach may be necessary, involving physicians, pain specialists, and therapists.
Psychological support and coping strategies can be helpful in managing the emotional effects of chronic pain.
Ongoing research is aimed at better understanding the causes and developing more effective treatments for erythromelalgia.