Erythromelalgia: Case Report in an Adolescent Girl

Erythromelalgia is a rare condition characterized by episodes of hyperthermia, erythema and pain in extremities, which may be provoked by heat or exercise. It is more common in the lower extremities but can also affect hands, nose and ears. Pathophysiology remains incompletely understood. Primary erythromelalgia it is more common in children and can be classified as acquired or inherited. Secondary type is very rare in pediatric age and is associated with other conditions, such as myeloproliferative disorders. The disease takes typically a chronic course and is associated with a decrease in the quality of life.

We present a case of a 15-years-old caucasian female patient. Previous personal medical history and familiar antecedents were unremarkable. She presented three times to the emergency department complaining of pain and erythema in the right hand, being always discharged to ambulatory and medicated with a short course of nonsteroidal anti-inflammatory drugs and referred to a paediatric consultation. During etiological investigation, the clinical picture evolved with the development of heat, redness and intermittent pain in both feet. She described spontaneous remission of symptoms with rest and immersion of the feet in water. Physical examination at the consultations was consistently without abnormalities. After 1 year of follow up, photos of the reported episodes revealed an erythema of toes and dorsum of the feet. Based upon recognition of characteristic signs, diagnose of erythromelalgia was made. At the present moment, remains on follow up at the pediatric consultation, treated with nonpharmacologic measures such us exposure of the affected area to cool water and avoidance of precipitants. In very painful crises she is medicated with pregabalin.

The present clinical case aims to raises awareness about this disease. Its early recognition will allow an accurate approach and a timely implemented treatment in order to reduce symptoms and improve quality of life.