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Cutaneous manifestations in erythromelalgia: causes, symptoms, diagnosis, treatment
Medical expert of the article
Last reviewed: 07.07.2025
Erythromelalgia is a disease that occurs as a result of vasomotor disorders.
Causes and pathogenesis. Depending on the origin, there are 3 types of erythromelalgia: type 1, associated with thrombocythemia, type 2 - primary, or idiopathic, which exists from birth, and type 3 - secondary, resulting from inflammatory and degenerative changes in blood vessels. In the development of erythromelalgia, a major role is played by a violation of the metabolism of vasoactive substances, in particular serotonin, vasodilation, etc. The disease is often familial. The presence of erythromelalgia in the fifth generation has been established.
Symptoms. The disease occurs equally often in men and women. Usually the arms and legs are affected, sometimes one limb. All types are characterized by exacerbations of the disease after a warm bath, exercise, even after sleeping in a warm bed. Patients complain of pain, burning, redness of the skin and increased temperature of the extremities. The height of the attack is manifested by purple-red or livid coloration, massive swelling and cyanosis of the skin of the hands, feet and shins. Attacks last from several minutes to several hours. With a long-term existence of the process, trophic disorders develop (hyperkeratosis of the palms and soles, paronychia) up to the formation of ulcers.
The course of the disease is chronic, febrile, lasting up to many years, with periodic intensification of the process.
Treatment. Vitamin therapy (B1, B12), antineuralgic, antihistamines, corticosteroids and angioprotectors (trentap, complamin) are prescribed, paravertebral blockade, sympathotomy, locally - adrenaline applications are performed.
Differential diagnosis. The disease should be distinguished from erysipelas, chronic atrophic acrodermatitis.
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