Erythromelalgia

Key Points
Skin condition which typically affects the hands and feet
Exact cause is unknown, but may develop in association with various disorders
Consists of severe pain, burning sensation, redness, and elevated skin temperature in the affected area
Pain is improved by cooling the region with a fan or ice bath

Erythromelalgia is a skin condition which typically affects the feet, but may also occur in the hands. Its pattern of effect may be bilateral or unilateral. In rare cases, the face, ears, or other areas may be affected. The condition presents as severe pain, a burning sensation, redness, and elevated temperature in the skin of the affected area. Symptoms may be continuous or present intermittently. Swelling, discoloration, mottling and tenderness may accompany flare-ups. Pain associated with the condition can be intense enough to reduce normal functionality. Pain is improved by cooling the region with a fan or ice bath.

The cause of erythromelalgia is unknown. Primary erythromelalgia develops idiopathically, unrelated to any disorder, while secondary erythromelalgia occurs in conjunction with neurological, autoimmune, and myeloproliferative diseases. Additionally, the condition may be genetic in some cases.

Differential Diagnosis (Other conditions with similar appearance)
Cellulitis
Frostbite
Reflex Sympathetic Dystrophy
Fabry disease
Peripheral neuropathy
Raynaud phenomenon
Vasculitis

Diagnosis
Key Points
Diagnosis based on appearance of the affected area in conjunction with clinical examination
Immersion testing may be performed to confirm diagnosis

Erythromelalgia is typically diagnosed based on the appearance of the affected area in conjunction with a clinical examination. Immersion testing may be performed to confirm the diagnosis.

Treatment
Treatment predicated on the type of the condition and the presence of an underlying disorder
Treatment consists of a combination of self-care, medications and surgical procedures
*Drug therapies vary in effectiveness

Treatment of erythromelalgia is often predicated on the type of the condition and the presence of an underlying disorder. Diagnosis and treatment of associated conditions is imperative. Treatment of the condition typically consists of a combination of various therapies, including self-care, drug therapies, and careful temperature regulation. It is recommended that affected persons keep affected areas cool and elevated to relieve symptoms. Topical treatments such as capsaicin preparations may also be useful. Drug therapies, which have varying rates of effectiveness, include aspirin, serotonin re-uptake inhibitors, tricyclic antidepressants, anticonvulsants, calcium antagonists, and prostaglandins. Other treatments which may be recommended include intravenous medications such as nitroprusside, lidocaine, and prostaglandin, and surgical sympathectomy, in which certain nerves are cut to relieve pain.