Refractory idiopathic erythromelalgia.

Erythromelalgia was first described by Mitchell in 1878 (1) and named from three Greek words: erythros (red), melos (extremities), and algos (pain). Through the years, the condition has been extremely rare, and Lewis (2), in 1933, felt that it lacked any precise criteria for definition and should be abandoned. Soon thereafter, Smith and Allen (3) reported a series of patients and described the condition in either primary or secondary idiopathic forms. Erythromelalgia can be characterized as a condition of painful, swollen, erythematous extremities. Intense burning pain and increased temperature of the involved. extremities are hallmark features. Although it is a disease of the extremities, the lower extremities are more frequently involved than the upper. Males are more frequently affected than females at a ratio of 2:1. Primary or idiopathic erythromelalgia occurs more frequently than secondary erythromelalgia, which is most commonly associated with a myeloproliferative disorder such as thrombocythemia or polycythemia vera (4). Other Conditions, such as myelogenous leukemia, hypertension, venous insufficiency, diabetes mellitus, systemic lupus erythermatosus, rheumatoid arthritis, lichen sclerosus et atrophicus, gout, spinal cord disease, multiple sclerosis, astrocytoma, and periucios anemia, have been found in association with secondary erythromelalgia (5-10). Primary erythromelalgia occurs most commonly in patients less than 30 years of age The etiology of primary erythromelalgia remained elusive. A defective prostaglandin metabolism may explain the red discoloration and burning pain seen in most patients (11). The following two case reports of idiopathic erythromelalgia in adolescent males demonstrate the need for aggressive techniques to manage the extraordinary pain symptomatology seen in these patients. These case reports are intended to provide an understanding of the pathophysiology of microvascular disturbances seen in these patients through the use of an Ice Cold Stress Test (ICST) and the protracted treatment course necessary to effect remission in Certain patients.