Erythromelalgia

Abstract

A 22‐year‐old woman has suffered for almost a year from bilateral erythema, swelling and warmth of hands and feet (Figure 1) progressing to severe pain. The pain was relieved by cold compresses several times a day and by elevating the affected extremities. For a few years she has had a recurrent respiratory infection, and 2 years before erythromelalgia (EM) symptoms she had infectious mononucleosis. A month before the onset of symptoms she had received an influenza vaccine with no unusual immediate reaction. All our patient's routine blood tests including platelet count were within normal limits. EM is a rare, poorly understood, chronic, sometimes progressive, and disabling disease that greatly affects the quality of life. EM is characterized by intermittent or constant intense burning pain, marked erythema and increased skin temperature, usually symmetrically on the lower extremities.1 When admitted to our department there was a symmetrical striking redness, swelling, tenderness, and warmth of the skin along with severe burning pain in distal limbs (Figure 1). At the beginning her symptoms appeared late during the day, but later on she had also these symptoms during the day, which were provoked by heat, exercise, and sometimes even by walking. The duration of her attacks lasted minutes to hours. The discomfort markedly affected her daily activities; she stopped hiking, playing sports, and even gave up her usual social activities. The diagnosis of EM was based on her medical history, clinical findings, and laboratory tests (hematological, biochemical, microbiological, immunological). Immunoglobulin G in enzyme‐linked immunosorbent assay test to Epstein‐Barr virus and cytomegalovirus was positive, while Immunoglobulin M was negative. Clinical and laboratory trials indicated that there was no systemic organ involvement. Peripheral pulses, tendon reflexes, neurological and electromyographic examination were normal. Plain radiography showed no specific findings of her feet and hands. Thermography revealed elevated skin temperature in the affected areas (hands and feet) and thermic amputation of the fingers on both hands (Figure 2). Treatment was extremely difficult. There is no consistently effective treatment for EM. Her symptoms were aggravated by heat and relieved by cold (ice). A multidisciplinary approach was necessary (instruction to the patient, learning how to avoid episodes, controlling secondary and underlying factors, use of drugs). At the beginning of The disease, aspirin was administered. Unfortunately, a few days after aspirin was administered, she developed an allergic drug eruption, which was confirmed by allergy testing. There was no improvement of her symptoms with combination of oral prostaglandin (misoprostol) in 800 mg daily dose and 10% capsaicin cream. Two months later, cyproheptadine (Periactin; Merck & Co., Whitehouse Station, NJ) 3 tablespoons daily (thereafter 2 tablespoons daily) was administered. Since she was using wet dressings frequently (a few hours a day for 5 months), she developed acrocyanosis on her hands and feet. The attacks were still present. Wet dressings during a few hours daily were useful. The symptoms were relieved upon cooling for the next few hours. For the time being, spontaneous remissions have not occurred. Since EM symptoms are still present, the patient tried seeking further help. Marked erythema and swelling producing tightness of both feet image Thermography. Elevated skin temperature in the affected areas and thermic amputation of the fingers on both hands. image