1/69. A case of erythromelalgia successfully controlled by systemic steroids and pentazocine--is it related to a unique subtype of neutrophilic dermatosis? erythromelalgia is a rare cutaneous disorder characterized by erythema, burning discomfort, and warmth of the extremities. Its form may be adult- or early-onset, and it may be further classified as idiopathic or secondary. Its pathogenesis and histopathologic findings have not yet been clearly defined. Various treatment modalities have been tried, but, for the idiopathic type, none has been successful. We present a case of adult-onset idiopathic erythromelalgia with unusual histopathological findings which showed a remarkable response to corticosteroids and pentazocine. ( info) |
2/69. A refractory case of secondary erythermalgia successfully treated with lumbar sympathetic ganglion block. A 59-year-old Japanese man with myasthenia gravis, who had a 10-year history of temperature-sensitive pain in the lower extremities, i.e. improved by cooling and worsened by warming, consulted us because the pain had become intolerable during the previous 4 months. Bilateral erythema, swelling and large ulcers were noted on the calves, dorsal aspects of the feet, and soles. Laboratory data showed thrombocythaemia and a positive antibody to the acetylcholine receptor, but were negative for antinuclear and antiphospholipid antibodies. A diagnosis of secondary erythermalgia was made because of the clinical features, the laboratory data, and the lack of family history of this disease. Although steroid pulse therapy, oral aspirin and antiserotonin drugs were ineffective, bilateral lumbar sympathetic ganglion block succeeded in relieving the severe pain and curing the ulcers. The clinical course in our patient suggests that sympathetic ganglion block may be one of the most effective treatments for secondary erythermalgia. Although the mechanism of this effect is uncertain, microcirculation disturbance in secondary erythermalgia, if any, may be improved by this block. ( info) |
| OBJECTIVE: The objective of this study was to determine if intractable pain from erythromelalgia could be successfully treated with intrathecal hydromorphone and clonidine. DESIGN: A single case of pain from erythromelalgia refractory to multiple treatment modalities was examined and treated. SETTING: The setting is an outpatient pain clinic at a major university teaching hospital. PATIENT: Our patient is an 82-year-old woman with hypertension and peripheral vascular disease. INTERVENTION: Intrathecal opioid and an alpha2-agonist were administered. OUTCOME MEASURES: Outcome was determined by means of patient self-report during office follow-up visits. RESULTS AND CONCLUSIONS: Administration of intrathecal opioid and an alpha2-agonist can be effective in the treatment of the pain of erythromelalgia and offers an alternative pain treatment modality for patients with unremitting pain refractory to more conservative therapy. ( info) |
| erythromelalgia is a clinical syndrome characterized by burning pain in the extremities together with erythema and increased skin temperature. Typically, the patients experience relief from cold, and aggravation from warmth. Symptoms are hypothesized to be caused by arteriovenous shunting and reduced nutritive skin capillary perfusion with corresponding tissue hypoxia. erythromelalgia is most often primary, but may be secondary to a wide variety of diseases. We report erythromelalgia in a patient with acquired immune deficiency syndrome (AIDS). At peak pain intensity he actively cooled hands and feet for more than 12 h/day. Many doctors handling human immunodeficiency virus/AIDS patients are unfamiliar with erythromelalgia, and the condition can easily be overlooked, especially the more common milder cases. ( info) |
5/69. Unexpected healing of cutaneous ulcers in a short child. A boy aged 10 years was referred to the Paediatric Department of Milan University Hospital, Milan, italy, with a long history of pain in the lower limbs, alleviated only by exposure to cold. His legs were swollen, with multiple cutaneous ulcers. He had severe painful crises, and was totally incapacitated. After the diagnosis of erythermalgia was made, numerous treatments were tried, but none were successful. After finding growth hormone (GH) deficiency, we started treatment with recombinant GH. He had immediate relief of pain and complete healing of ulcers. We postulate that the healing of the ulcers can be attributed to the GH-promoting effect on dermal connective tissue. ( info) |
| OBJECTIVE: To report the first European observations of erythromelalgia due to mushroom poisoning. methods: Clinical features of erythromelalgia were observed in 7 cases seen over 3 years. All patients had eaten the same mushrooms species, gathered in the same French alpine valley. erythromelalgia was first described in japan after Clitocybe acromelalga ingestion. Clitocybe amoenolens was identified as the possible cause of poisoning in our cases. ( info) |
7/69. Resolution of refractory symptoms of secondary erythermalgia with intermittent epidural bupivacaine. BACKGROUND AND OBJECTIVES: Erythermalgia is a rare disorder characterized by erythema, edema, elevated skin temperature, and burning pain of the hands and/or feet. The etiology of primary erythermalgia is idiopathic while secondary erythermalgia is associated with autoimmune and rheumatologic factors. Symptoms are typically refractory to medical management. We report the use of epidural bupivacaine as a treatment option for secondary erythermalgia. CASE REPORT: A 28-year-old woman presented with secondary erythromelalgia that was characterized by severe pain, swelling, and erythema. Medical management failed and a lumbar epidural dosed with bupivacaine was used. This resulted in complete resolution of the patient's symptoms. CONCLUSION: Intermittent epidural bupivacaine may provide treatment of the refractory symptoms of secondary erythermalgia. ( info) |
| We describe two unrelated children with almost identical clinical illnesses comprising of severe, burning dysesthesia, allodynia, hypertensive encephalopathy, and laboratory evidence of both sympathetic and parasympathetic autonomic disturbance after a nonspecific viral illness. No underlying etiology was identified. Both patients displayed complete resolution of their clinical and radiologic findings after a number of months, and there was no recurrence over a follow-up period of 17 months to 4 years. Treatment of the patients' dysesthesias proved difficult, requiring multiple analgesics and intensive physiotherapy. We speculate that their illnesses may represent a pure autonomic variant of guillain-barre syndrome. ( info) |
9/69. High-dose oral magnesium treatment of chronic, intractable erythromelalgia. BACKGROUND: Previous studies with magnesium have shown beneficial effects in pain syndromes and in vascular disorders (hypertension, migraines, Raynaud's phenomenon). However, results have been variable, possibly because of the limited oral doses achievable due to frequent diarrhea. OBJECTIVE: To describe the case of a 53-year-old white man (the author) with disabling erythromelalgia (EM) who achieved modest improvement limited by adverse effects with calcium antagonists and then obtained remission with the use of magnesium. Intolerant of several standard magnesium products, he attained high doses of magnesium by taking intravenous-grade magnesium sulfate diluted in water orally (up to 24 mL/d of MgSO4 50% = 1166 mg/d of magnesium). methods: After 12 months of continued improvement, patient 1, a board member of The erythromelalgia association, notified other members of the success of this therapy via an internet communication channel. He encouraged those interested in high-dose magnesium therapy to consult their physicians and to report their results to the association. Twelve patients responded to this request, describing several standard oral magnesium products. RESULTS: overall, 8 of 13 patients (61.5%) reported improvement (1, remission; 3, major improvement; 2, moderate improvement; 2, mild improvement). Four patients (30.8%) reported no response to magnesium therapy, and 1 patient's symptoms worsened. Two patients' magnesium dose was limited because of diarrhea. CONCLUSIONS: Despite recent progress in understanding and treating EM, this vascular disorder remains painful and life-altering for many patients. In this informal survey, the use of high oral doses of magnesium produced good and sometimes dramatic results in 8 of 13 patients who had been unresponsive to many other treatments. These results suggest a possible role for high-dose oral magnesium in the treatment of EM and, perhaps, other vascular disorders. ( info) |
| erythromelalgia is an unusual syndrome of painful vasodilatation. Aetiopathology is probably different in children and adults. Presentation can be severe and associated with hypertension. Dramatic benefit from infused nitroprusside suggests the disorder could represent a dysfunctional endothelium. ( info) |