Erythromelalgia is a rare disease existing in two forms: primary and secondary (Cohen 2000). Primary erythromelalgia is the most common form and can begin at any age. Secondary erythromelalgia has been reported with many disorders, most commonly polycythemia and myelodysplastic disorders. It has also been associated with connective tissue disorders, most commonly SLE.
Erythromelalgia is characterized by prominent erythema and increased temperature involving predominantly the feet, but the hands also may be involved. Characteristically intense burning pain is present. The condition may be bilateral but at times may involve only one extremity. Exposure to warmth frequently triggers an attack. Ice water immersion of the affected extremity produces pain relief.
The etiology of erythromelalgia is unknown (Cohen 2000). However, recent research suggests an imbalance in limb perfusion characterized by some precapillary sphincter constriction while at the same time arteriovenous shunts remain open. It is theorized that this combination of vascular abnormalities produces the coexistence of hypoxia and hyperemia in the affected extremity.
This is a rare complication of SLE. Alarcon-Segovia et al. (Alarcon-Segovia et al. 1963) reviewed 51 cases of erythromelalgia at the Mayo Clinic between 1951 and 1960. Only one of these 51 cases had classic manifestations of SLE.
A recent study of chilblain LE indicated that 3 of 22 patients also had erythrome-lalgia (Viguier et al. 2001). Other vascular abnormalities, such as acrocyanosis and Raynaud's phenomenon, were prominent in this group of patients. This study also suggests that erythromelalgia in patients with SLE occurs in the setting of temperature-dependent vascular insults.
The treatment of erythromelalgia is problematic. Aspirin, prostaglandins, tetra-cycline, antidepression drugs inhibiting serotonin reuptake, calcium antagonists, nitroprusside infusions, lidocaine patches, and infusions have all been tried with varying degrees of success.
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