Defining a treatable cause of erythromelalgia: acute adolescent autoimmune small-fiber axonopathy.
نویسندگان
چکیده
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.
منابع مشابه
Erythromelalgia: an underrecognized manifestation of small-fiber neuropathy.
Erythromelalgia is characterized by intermittent heat, redness, and pain affecting the extremities.1 Exercise and increase in temperature are precipitating factors. During symptoms, blood flow and temperature increase without a concomitant increase in oxygenation.2 Severe sudomotor impairment and evidence of peripheral adrenergic dysfunction are also present.2 A recent study showed that a predo...
متن کاملThermoregulatory sweat testing in patients with erythromelalgia.
OBJECTIVES To examine the results of thermoregulatory sweat testing in patients with erythromelalgia and to compare them with the results of other neurophysiologic tests of small-fiber nerve function. DESIGN Retrospective study. SETTING Tertiary referral center. PATIENTS Thirty-two consecutive patients with erythromelalgia who had thermoregulatory sweat testing in addition to vascular and...
متن کاملO 18: Autoimmune Dementia
Dementia is defined as significant acquired cognitive impairment in one or more cognitive domains that represents a significant decline from previous baseline and interferes with independence in daily activities. Autoimmune dementia is a term that has been used to describe a steroid-responsive autoimmune disorder characterized by a rapidly progressive dementia with a fluctuating course. Even co...
متن کاملInitiation and progression of axonopathy in experimental autoimmune encephalomyelitis.
Axonal loss is the principal cause of chronic disability in multiple sclerosis and experimental autoimmune encephalomyelitis (EAE). In C57BL/6 mice with EAE induced by immunization with myelin oligodendrocyte glycoprotein peptide 35-55, the first evidences of axonal damage in spinal cord were in acute subpial and perivascular foci of infiltrating neutrophils and lymphocytes and included intra-a...
متن کاملPathological nociceptors in two patients with erythromelalgia‐like symptoms and rare genetic Nav 1.9 variants
INTRODUCTION The sodium channel Nav 1.9 is expressed in peripheral nociceptors and has recently been linked to human pain conditions, but the exact role of Nav 1.9 for human nociceptor excitability is still unclear. METHODS C-nociceptors from two patients with late onset of erythromelalgia-like pain, signs of small fiber neuropathy, and rare genetic variants of Nav 1.9 (N1169S, I1293V) were a...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید
ثبت ناماگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید
ورودعنوان ژورنال:
- Anesthesia and analgesia
دوره 104 2 شماره
صفحات -
تاریخ انتشار 2007