A rare case of facial erythromelalgia: a promising way of treatment with combination of intense pulsed light and plasmapheresis

Abstract

Erythromelalgia is a rare disorder characterized by episodic burning pain, erythema, and increased temperature. Most often, the pathological process affects the distal extremities. There are primary (hereditary) and secondary forms of the disease. It is considered that erythromelalgia is caused by abnormal vascular dynamics, in which capillary perfusion decreases, provoking tissue hypoxia. At the same time, there is an increase in arteriovenous shunting in the skin, which therefore appears erythematous, warm, and edematous. There are no universally effective protocols for the treatment of erythromelalgia, so the management of such patients is largely difficult. A 32‑year-old patient came to us with complaints of paroxysms of erythema, burning pain, and fever in the cheeks, nose, and ears. In addition to centrofacial erythema and subjective sensations of pain and burning, the patient's fingers turned white in the cold, then acquired a cyanotic color and, finally, a bright red color. Color changes were accompanied by pain sensations of tingling and burning. After a comprehensive examination of the patient by a rheumatologist, we assumed a rare diagnosis of isolated facial erythromelalgia. A combination of 2 intense pulsed light treatments (M22 Lumenis) and 3 plasmapheresis sessions (Haemonetics MCS) was successfully used as therapy. The manifestations of erythema significantly decreased, the intensity of facial burning reduced, and the titer of antinuclear factor normalized. The patient was satisfied with the results of the treatment. The article emphasizes the importance of managing patients with erythromelalgia by a multidisciplinary team of specialists, which ensures both a thorough differential search and verification of the diagnosis, and an integrated approach to therapy. In the absence of a universal and effective method for treating this pathology, the proposed therapy regimen may be of particular interest to clinicians of various specialties who are faced with such cases.