Erythromelalgia

Erythromelalgia in hands of a Scandinavian male, 52, after holding a book).

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Toes during an EM flareup

Sir Thomas Barlow

Erythromelalgia

Erythromelalgia, formerly known as Mitchell's disease (named after Silas Weir Mitchell), is a rare vascular peripheral pain disorder. This condition leads to the episodic blocking of blood vessels, typically in the lower extremities or hands, which subsequently become inflamed and hyperemic.

Definition

Erythromelalgia is a disorder characterized by:

• Intense burning pain.
• Redness (erythema).
• Increased temperature in the affected areas.
• Episodes of symptom exacerbation and relief.

History

The condition was first described by Dr. Silas Weir Mitchell in the 19th century, hence the former name Mitchell's disease.

Pathophysiology

The exact cause of erythromelalgia remains unclear, but it is believed to result from:

• Abnormalities in the normal vasomotor responses or functioning of the blood vessels.
• Possible genetic mutations in some cases.

Symptoms

Main symptoms include:

• Intense burning or throbbing pain.
• Swelling or inflammation of the affected region.
• Increased warmth and redness in the affected areas.
• Episodes that may last from minutes to hours or even longer.

Diagnosis

Diagnosis is primarily clinical, based on:

• Detailed medical history.
• Physical examination.
• Exclusion of other conditions.

In some cases, a skin biopsy or other tests might be performed to rule out other disorders.

Treatment

There is no definitive cure for erythromelalgia, but treatments aim to relieve symptoms. Common approaches include:

• Cooling the affected area.
• Avoiding triggers such as heat or specific activities.
• Pain relievers like NSAIDs or aspirin.
• Topical creams or patches.
• Certain anticonvulsants or antidepressants.

Epidemiology

Erythromelalgia is a rare condition, with a variable reported incidence. It can affect people of all ages but may be more common in certain age groups.

See Also

• Vascular disorders
• Pain disorders
• Peripheral neuropathy
• Silas Weir Mitchell

External links

Classification	ICD-10: I73.8 ICD-9-CM: 443.82 OMIM: 133020 MeSH: D004916 DiseasesDB: 4476
External resources	eMedicine: article/200071

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