Erythromelalgia (EM) is a rare disease characterized by recurrent episodes of erythema, pain, and burning dysesthesia, most often involving the extremities. A rare, atypical presentation involves the face. There are no current guidelines, with overall lack of consensus for the treatment of EM, and therapies are often formulated on a case-by-case basis. We describe the case of a 48-year-old woman who presented with facial EM causing erythema and burning pain with significant impact on quality of life, and was successfully treated with stellate ganglion block. This case highlights that stellate ganglion blocks can be an effective treatment option for patients with facial EM, alongside other conservative therapies that may include medical management.
Paticoff J, Valovska A, Nedeljkovic SS, Oaklander AL
Anesthesia and analgesia •
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.
