Erythromelalgia is a chronic angiotrophoneurosis, manifested by paroxysms of local burning pain with swelling and sharp redness of the skin. As a rule, it occurs in the feet, less often in the hands, the area of the auricles, nose. It can have a primary and secondary character. Pathology is diagnosed according to a typical seizure clinic. Additionally, laboratory tests, radiography, thermography, CT, MRI, electrophysiological studies are carried out. Complex therapy includes vasoconstrictors, antihistamines, anti-inflammatory medications, acupuncture and physiotherapy.
ICD 10
I73.8 Other specified peripheral vascular diseases
General information
The disease was first described by N.I. Pirogov in 1855 as a result of observations of wounded soldiers. Then the attacks of erythromelalgia were given the name “posttraumatic hyperesthesia”. A decade later, surgeon Mitchell, observing the victims of the war in the United States, revealed similar symptoms in some. He introduced the term “erythromelalgia”, which turned out to be more successful and is still used by clinicians. Indeed, the name contains the whole essence of the bright clinical manifestations of the disease. Translated from Latin: “erythros” — “red”, “melos” — “limb”, “algos” — “pain”. In literary sources, it is sometimes referred to as Mitchell’s disease.
Pathology is among the rare. Along with Raynaud’s disease, it refers to angiotrophoneurosis — disorders of peripheral circulation associated with impaired vascular innervation. As a rule, it is local in nature with the involvement of one or both feet. Possible damage to the hands. It occurs more often in people 30-50 years old, in children — in isolated cases. The incidence has no gender differences.
Erythromelalgia causes
- Primary erythromelalgia acts as an independent disease. Secondary can be observed in a number of pathological conditions:
- spinal cord lesions (myelitis, syringomyelia, multiple sclerosis, neurosyphilis);
- blood diseases (polycythemia, essential thrombocytemia);
- alcoholism;
- endocrine diseases (hypothyroidism, diabetes mellitus, hypercorticism syndrome);
- metabolic disorders (gout);
- vibration disease.
Pathogenesis
Etiopathogenesis has not been definitively studied. One thing is clear — the disease is based on a disorder of the nervous regulation of the vascular wall. The result of a violation of vasomotor innervation is a local dilation of peripheral vessels of small and medium caliber. There is a restructuring of the blood flow in the direction of increasing the flow of arterial blood through arterio-arterial and arterio-venous messages. Clinically, this is manifested by redness.
Vascular dilatation is accompanied by an increase in the permeability of the vessel wall, which entails edema. The emergence of pain syndrome is probably caused by the release of some humoral factors from the vascular bed and the overexpression of sympathetic fibers. The level of damage to the nervous system, as a result of which erythromelalgia occurs, has not yet been established. These may be pathological processes in the posterior horns of the spinal cord, spinal roots and trunks of peripheral nerves.
Symptoms
The basis of the clinical picture is a typical erythromelalgic paroxysm (attack). Burning or baking pain (sympathalgia), accompanied by intense redness and swelling. In many patients, these signs appear first in the area of the big toe, and then spread to the entire foot and the second leg. Pain attacks can occur in two legs at the same time, in the hands; in some cases — in the nose, ears, perineum, in women, breast damage is possible. The pain is so pronounced that during the period of paroxysm, the patient often cannot walk.
Seizures are provoked by overheating of the limb, its hanging position, compression (for example, wearing tight shoes). Some patients associate the onset of paroxysm with wrapping and squeezing the legs with a blanket. The duration of paroxysms varies from 2-3 minutes to several hours. Objectively, during the attack, there is marked redness of the skin of the affected area, swelling and a palpable increase in local temperature. Warming the affected limb, walking and squeezing sharply increase the pain, the cold helps to reduce it. The elevated position of the limb facilitates the patient’s condition, since it reduces blood flow.
Mitchell’s disease is characterized by a chronic persistent course. In the hot season, the frequency of attacks increases significantly. Progression is often noted — paroxysms become more frequent and become more prolonged, their severity increases, erythromelalgia captures an increasingly large area, spreads symmetrically to the second leg, and then to the hands. In the interparoxysmal period, vascular and trophic changes are noted in the affected area. There is cyanosis and dryness of the skin, brittle nails, the formation of ulcers is possible. The swelling takes on a permanent character. Paresthesia, hypersensitivity (hyperesthesia) or distortion of sensory perception (hyperpathy) are noted.
Diagnostics
A typical clinical picture allows a neurologist to establish a diagnosis without difficulty. However, differential diagnosis with other similar diseases and identification/exclusion of the underlying pathology is important. The presence of sympathalgia is the main clinical difference between Mitchell’s disease and acroerythrosis. The latter occur in damp and cold climates, and erythromelalgia, on the contrary, gives an improvement in cool weather. Diphdiagnosis with Raynaud’s disease, thrombophlebitis, deep vein thrombosis, arthritis of the joints of the foot, erysipelas is also necessary. The secondary nature of erythromelalgia is indicated by unilateral localization of changes.
- Diagnosis of causal pathology is performed by thermography, radiography of the foot, ultrasound of the vessels of the lower extremities, capillaroscopy, MRI of the lumbar spine.
- The study of peripheral nerve trunks is carried out using ultrasound of the nerve and electroneuromyography.
- According to the indications, a CT or MRI of the foot is performed.
- Of no small importance is a general blood test (detection of inflammatory changes), a biochemical blood test (determination of glucose, uric acid, rheumatoid factor), a study of the level of cortisol and thyroid hormones.
- If an endocrine pathology is suspected, the patient is referred to an endocrinologist, if vascular disorders are detected during the examination, to a phlebologist or vascular surgeon.
Erythromelalgia treatment
Conservative therapy
Complex therapy with the use of medications and physiotherapeutic agents is carried out. Since the main pathogenetic mechanism of attack in Mitchell’s disease is vasodilation, vasoconstrictive pharmaceuticals are used during paroxysm: mezaton, adrenaline, ephedrine. Their use requires caution, since it is associated with the risk of developing arterial hypertension.
Antihistamines are used as decongestant therapy: clemastine, chloropyramine. Pain relief is carried out with the help of NSAIDs: ibuprofen, diclofenac, novocaine blockades are carried out in severe situations.
The complex treatment includes pharmaceuticals that increase the strength of the vascular wall (ascorbic acid, rutin, calcium preparations). In the interparoxysmal period, reflexotherapy sessions, physiotherapy (darsonvalization, mud therapy, foot and hand baths), sanatorium treatment (radon, carbon dioxide sources) are prescribed.
Surgical treatment
Erythramelalgia with severe and poorly relieved pain syndrome is an indication for surgical treatment. Sympathectomy is performed — removal of sympathetic nodes responsible for innervation of the affected area. The operation does not lead to the disappearance of paroxysms, but allows you to relieve the patient from the excruciating pain syndrome.
Prognosis and prevention
The disease is not life-threatening, but the prognosis for recovery is doubtful. The secondary form has a more favorable course, with the elimination of causal pathology, a cure is possible. Primary erythromelalgia is characterized by a more severe and progressive course with an expansion of the zone of pathological changes and an increase in the frequency of attacks. The literature describes isolated cases of spontaneous recovery.
Secondary prevention consists in the exclusion of factors provoking attacks: physical overstrain, overheating, wearing tight shoes. When working with muscle tension, being in hot rooms, patients need to change their professional activities.
