Erythromelalgia involving the face

Abstract

Erythromelalgia is a rare disorder characterized by burning pain, erythema, and increased temperature typically involving the distal extremities. Although it can progress to involve the face, erythromelalgia presenting only on the face is particularly rare. This disorder is often misdiagnosed when it presents on the extremities and is even more likely to be misdiagnosed when presenting only on the face, delaying appropriate treatment and causing considerable frustration for the patient. We report a case of a 26-year-old woman with erythromelalgia that involved only the face for a number of years and was treated unsuccessfully as rosacea, seborrheic dermatitis, and contact dermatitis. She subsequently developed involvement of the ears and hands in the more typical distribution of erythromelalgia. We discuss the differential diagnosis of erythromelalgia involving the face and extremities, the proposed pathogenesis and management of the disorder, and the psychological distress this condition can cause. Even when the correct diagnosis of erythromelalgiais made, treatment is difficult, with no single therapy consistently effective.