Abstract

Erythromelalgia is a rare disorder characterized byburning pain, erythema, and increased temperaturetypically involving the distal extremities. Althoughit can progress to involve the face, erythromelalgiapresenting only on the face is particularly rare. Thisdisorder is often misdiagnosed when it presentson the extremities and is even more likely to bemisdiagnosed when presenting only on the face,delaying appropriate treatment and causingconsiderable frustration for the patient. We report acase of a 26-year-old woman with erythromelalgiathat involved only the face for a number of years andwas treated unsuccessfully as rosacea, seborrheicdermatitis, and contact dermatitis. She subsequentlydeveloped involvement of the ears and hands inthe more typical distribution of erythromelalgia. Wediscuss the differential diagnosis of erythromelalgiainvolving the face and extremities, the proposedpathogenesis and management of the disorder, andthe psychological distress this condition can cause.Even when the correct diagnosis of erythromelalgiais made, treatment is difficult, with no single therapyconsistently effective.