Erythromelalgia

Abstract

Erythromelalgia is an extraordinary disorder of unknown etiology and pathophysiology that resembles the post-traumatic reflex dystrophy syndromes but has not been described previously in the orthopedic literature. Its distinctive triad of intense burning extremity pain associated with erythema and increased skin temperature are diagnostic. Primary or idiopathic and a secondary or associated form have been identified. The latter occurs in association with an underlying disease process, especially myeloproliferative disorders. Treatment with pharmacologic agents and surgery are ineffective except in the secondary group where treatment of the associated disorder generally results in a remission. Symptoms in the primary group can be minimized by appropriate environmental control with cooling and avoiding heat-producing situations that would raise skin temperature above a critical thermal threshold.