Manifestations in the Oral Mucosa of Erythromelalgia. A Case Report

Erythromelalgia or erythermalgia, is a peripheral paroxysmal vascular disease of the distal extremities, although it can also affect the face, ears and knees. It is characterized by increased skin temperature, erythema and intermittent episodes of burning pain. Likewise, it can also be characterized by lesions of the oral mucosa.

Here we present the case of a 6-year old female with EM presenting ulcerative and scaly lesions in the oral cavity and whitish linear branching ulcerative lesions on the tongue. Fabry disease and rheumatoid conditions were ruled out and there were no mutations in SCN9A gene. Treatment with voltage-gated sodium channel blockers (oxcarbacepine-Trileptal^®) and anticonvulsants (Perampanel-Fycompa^®) did not improve pain symptoms, however, performing moderate physical activity, use of comfortable footwear, and local application of cold resulted in the restoration of patient´s quality of life

To our knowledge, this report described for the first time, the successful treatment of ulcerative lesions of the oral mucosa in Erythromelalgia. We believe that the treatments described here (exercise, comfortable footwear and local application of cold) could improve the quality of life of Erythromelalgia patients.