Defining a treatable cause of erythromelalgia: acute adolescent autoimmune small-fiber axonopathy.
Conditions described as "erythromelalgia" and "erythermalgia" are being formally specified by etiological diagnoses that enable the use of disease-modifying as well as symptomatic treatments. We describe an otherwise healthy 20-year-old man with acute-onset erythromelalgia. Severe bilateral distal limb pain and vasodilation persisted despite the use of many antihyperalgesics. Pathological examination of cutaneous nerve endings revealed severe small-fiber predominant axonopathy. Treatment of his apparent autoimmune polyneuropathy with high dose corticosteroids, 4 days of lidocaine infusion, and a prednisone taper cured him. Similarities to other cases allowed us to tentatively characterize a new treatable cause of erythromelalgia; acute adolescent autoimmune small-fiber axonopathy. In this report we evaluate various options for diagnosis and treatment.
Paticoff
Joshua
J
Department of Anesthesiology, Perioperative and Pain Medicine, Brigham and Women's Hospital, Boston, Massachusetts, USA.
Valovska
Assia
A
Nedeljkovic
Srdjan S
SS
Oaklander
Anne Louise
AL
D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000700:Analgesics / Q000627:therapeutic use; D001369:Axons / Q000473:pathology*; D004916:Erythromelalgia / Q000209:etiology* / Q000473:pathology / Q000628:therapy*; D020275:Guillain-Barre Syndrome / Q000150:complications* / Q000473:pathology / Q000628:therapy*; D006801:Humans; D008297:Male; D009411:Nerve Endings / Q000473:pathology*
D000305:Adrenal Cortex Hormones; D000700:Analgesics