Description:

“... features of erythromelalgia include lower extremity vasodilatation, redness, burning pain and increase in temperature.”

Synonyms: Erythromelalgia, erythermalgia

Classification and Types:

Primary: sometimes reported as an autosomal dominant but most likely idiopathic

Secondary: associated with other disorders such as myeloproliferative disorders and thrombocytosis.

Smith and Allen in 1938 used the term erythermalgia to place an emphasis on the warmth noted with this disorder. They were the first to subdivide the disorder into primary and secondary.

Two differing systems of classification exist today. Drenth and Michiels (1994, 1995) proposed using the terms erythromelalgia for a platelet-mediated form that is responsive to aspirin and use the terms primary and secondary erythermalgia for non-aspirin sensitive forms of the disorder.

Kurzrock and Cohen (1991) subdivide the disorder into adult-onset idiopathic and adult-onset secondary along with early-onset idiopathic and early-onset childhood forms. The early onset form has a male to female ratio of 1:2.5. Secondary erythromelalgia caused by myeloproliferative disorders seem to occur in a male to female ratio of 3:2.

Description:

Erythromelalgia is a rarely seen clinical disorder. The name of this clinical entity comes from the Greek for red (erythros), extremity (melos), and pain (algos).

Erythromelalgia is most commonly seen in the secondary form. This may usually occurs in conjunction with myeloproliferative disorders. Along with the myeloproliferative disorder other hematological abnormalities such as polycythemia and thrombocytosis are often seen. Studies have reported erythromelalgia to occur in from 3 to 65% of patients with myeloproliferative disorders. A comprehensive listing of causes of secondary erythromelalgia would include: polycythemia vera, essential thrombocythemia, myelofibrosis and chronic myelogenous leukemia. Some reports have also ascribed this condition to being related to rheumatoid arthritis, gout, vasculitis, pernicious anemia, SLE and possibly drug induced (bromocriptine, nicardipine, nifedipine, pergolide and verapamil).

The features of erythromelalgia include lower extremity vasodilatation, redness, burning pain and increase in temperature. This may last from minutes to days or longer. Itching may accompany the other symptoms.

The soles of the feet and toes are most often affected, but it may affect the lower extremity up to the knee. I have seen this occur clinically in conjunction with a myeloproliferative disorder.

The symptoms of erthromelalgia often occur up to 2.5 years before the myeloproliferative disorder is diagnosed.

In cases with thrombocythemia the platelets have a decreased time of survival and hyperaggregability. Prostaglandins and cyclooxygenase seem to play a vital role in the pathogenesis of this disorder.

Diagnostic Studies:

It is important to rule out myeloproliferative disorders. A CBC and differential should be performed. Look for a high platelet count and/or a high hematocrit (larger then 50%).

Differential Diagnosis:

Complex Regional Pain Syndrome
Shoulder-Hand Syndrome
Fabry’s disease
Cellulitis

Reported Therapy:

Intermittent ice application, daily aspirin (500 mg po each day), elevation.

Chemotherapy to lower platelet count.

Phlebotomy in patients with polycythemia vera.

Avoid over-warming the extremities. Elevate legs often throughout the day.

References and additional resources:

Drenth JP, van Genderen PJ, Michiels JJ: Thrombocythemic erythromelalgia, primary erythermalgia, and secondary erythermalgia: three distinct clinicopathologic entities. Angiology 1994 Jun; 45(6): 451-3[Medline].

Merc Manual. Erythromelalgia. http://www.merck.com/pubs/mmanual/section16/chapter212/212f.htm

Michiels JJ, Abels J, Steketee J: Erythromelalgia caused by platelet-mediated arteriolar inflammation and thrombosis in thrombocythemia. Ann Intern Med 1985 Apr; 102(4): 466-71[Medline].

Michiels JJ, Drenth JP, Van Genderen PJ: Classification and diagnosis of erythromelalgia and erythermalgia. Int J Dermatol 1995 Feb; 34(2): 97-100[Medline].
Nardino, RJ, et. al.. Erythromelalgia: eMedicine Topics. http://www.emedicine.com/med/topic730.htm

Organizations:

NIH/National Institute of Arthritis and Musculoskeletal and Skin Diseases
1 AMS Circle
Bethesda, MD 20892-3675
Tel: (301)496-8188
Fax: (301)718-6366
Tel: (877)226-4267
TDD: (301)565-2966
Email: NAMSIC@mail.nih.gov
Internet: http://www.nih.gov/niams/

Erythromelalgia Association
4343 Roosevelt Way, NE
#305
Seattle, WA 98105
Tel: (206)632-0894
Fax: (206)632-1894
Email: jeanmilt@prodigy.net
Internet: http://www.erythromelalgia.org