Erythromelalgia: causes, symptoms, treatment

Erythromelalgia (synonyms: Mitchell's syndrome, painful limited edema of Pospelov's skin, acromelalgia, Lannoy's vasomotor paralysis) is quite rare disease of the autonomic nervous system, the clinical signs of which arise from the paroxysmal enlargement of small arteries on the hands and feet. As a result, acute burning pain in the extremities appears, reddening of the skin of the hands and feet develops, the temperature rises locally (hands and feet become hot to the touch).

Clear reasons for the onset of the disease are still a mystery to the medical profession. Diagnosis is based on the characteristic clinical signs of painful attacks. Treatment of the disease, in the first place, is conservative and consists of medicinal action and physiotherapy techniques. In cases that are not amenable to conventional therapy, resort to surgical methods of treatment. From this article you can learn more about the causes, symptoms, methods of treatment of this disease.

Causes of erythromelalgia

The doctors do not yet know the only true cause of the disease. Medicine knows only that erythromelalgia can be an independent disease, and maybe manifestation of other pathological conditions (in this case erythromelalgia is, as it were, secondary). Among other diseases, the manifestation of which it may be, the following are known:

• syringomyelia;
• multiple sclerosis;
• dorsal;
• diabetes;
• blood diseases (especially polycythemia);
• consequences of injuries, including frostbite;
• pronounced osteochondrosis of the spine;
• myxedema;
• phlebitis;
• endarteritis;
• alcoholism;
• gout.

Erythromelalgia, which is an independent disease, usually proceeds more severely. Secondary erythromelalgia has a more favorable course and is more easily tolerated by patients.

At the heart of the emergence of clinical manifestations of erythromelagia is the pathology of various levels of the sympathetic nervous system. As a result, there are violations of blood microcirculation in the peripheral parts of the limbs. Through arteriovenous anastomoses, a large amount of blood rushes, which is accompanied by vasodilation, causes a local increase in temperature, the skin becomes hot and turns red. Since the vessels have a rich innervation of sympathetic nerve fibers, their redistribution by excessive blood flow causes the appearance of a strong pain of a burning, baking character. Reflex spasm of blood vessels in order to reduce the flow of blood in erythromelalgia does not occur, which is also due to the pathology of the sympathetic structures of the nervous system. As a result, the attack of erythromelalgia lasts for a while, until the blood flow decreases.

Symptoms

Attacks of erythromelalgia are characterized by burning pain in the extremities and their reddening.

Erythromelalgia is a disease of people mostly young. According to statistics, male sex is more prone to primary disease than female.

Erythromelalia has a paroxysmal course. Periodically, seizures occur with characteristic features. The main clinical symptoms are:

• local redness of the skin of the hands and / or feet;
• increased temperature of the affected limbs at the time of the attack;
• burning, baking, unbearable pain in the hands and feet;
• increased sweating of the affected limbs at the time of attack.

Similar seizures last from several minutes to several hours, sometimes up to several days. The occurrence of an attack can be spontaneous, and can be provoked by some factors, for example, overheating (both locally and in general by increasing the temperature of the environment), physical activity. Patients may not wear socks, they do not cover themselves with a blanket, do not wash their limbs in warm water, so as not to provoke the appearance of pain. Summer hot weather becomes unbearable for such patients, as it causes more frequent attacks of pain. Characterized by a more frequent occurrence of attacks in the evening and at night. The attack ends on its own. The interictal period lasts from 10-15 minutes to several weeks.

Attacks of erythromelalgia do not necessarily capture limbs, although this happens most often. An attack can involve the tip of the nose, earlobes, a finger of a limb, a heel, a sole. One-sided process more often then becomes bilateral, and the zone of pain and redness expands. In the beginning of the disease the symptoms are disturbed for a short time, for example, from a few minutes to several hours, then last all night, and as the disease progresses, seizures last for days. Pulsation of arteries during an attack of erythromelalgia is preserved, which is used as a criterion for differential diagnosis with obliterating endarteritis. For erythromelalgia, the following feature is characteristic: the longer a disease exists, the more pronounced it is manifestations of both the prevalence of the process, and the duration of seizures and the power of pain. Fortunately, this rule only works if there is no treatment for the disease.

Touching the affected area during an attack causes an increase in pain. Relief to patients brings ice water, in which they try to immerse the limbs. And if the lower extremities are affected, then some of the patients in winter simply take off their shoes and walk barefoot in the snow to ease their condition.

A typical attack of erythromelalgia in the primary form of the disease, or, as it is also called, erythromelagic crisis, proceeds approximately like this: the hands and feet suddenly become hot and red, sometimes even with a cyanotic shade. In these places there is a burning, unbearable, throbbing pain. Affected limbs swell, become wet from sweat, there may be a small-dot rash in the area of ​​redness. Patients can not find a place, they intuitively raise their limbs upwards to reduce the flow of blood, and accordingly, pain, or lower them into cold water. Often patients take the supine position, as this also alleviates the pain somewhat. Because the pain is very strong, the attack has a pronounced emotional color and is hard to tolerate. At the end of the attack (which happens spontaneously after a while), the swelling of the limbs gradually decreases, the natural color and skin temperature returns. And after a few hours, there is not a trace left of the changes.

When the disease already has a long record of service, it is possible to develop persistent trophic disorders of the affected areas, which remain outside the attack. The skin thickens, it becomes dry and rough, the nails also thicken, become brittle and cloudy, the fingers can be thickened and deformed. But trophic ulcers do not develop with this disease.

Secondary erythromelalgia proceeds more benignly, if so it is possible to express. The seizures themselves are less severe in the strength of pain, shorter, and trophic disturbances even in the long course of the disease are not very pronounced. Usually, the affected area is stationary, that is, the process does not spread to another area or adjacent limbs. When examining such patients, the underlying disease is identified, which causes the occurrence of attacks of erythromelalgia.

Treatment

After the diagnosis of erythromelalgia is established, it is necessary to find out whether it is primary or secondary. Secondary erythromelalgia requires, first of all, the treatment of the disease that caused it, and the second - the symptomatic treatment of the clinical manifestations of erythromelalgia themselves.

Treatment must necessarily be comprehensive. They always begin with conservative measures: with the use of medications along with physiotherapy techniques. With ineffectiveness of such measures, it is possible to resolve the issue of surgical treatment.

From medicines usually use:

• vasoconstrictive drugs (because they reduce blood flow through arteriovenous anastomoses, thereby reducing stretching of the walls of the vessels and irritation of sympathetic fibers): Mesaton, Ephedrine, Ergotamine, Adrenaline. With these drugs, you should be careful, as they provoke an increase in blood pressure;
• tonic nervous system drugs: Caffeine, Phenamine;
• vitamins: Ascorbic acid, Rutin, Cyanocobalamin (vitamin B12);
• preparations of calcium;
• acetylsalicylic acid;
• anticonvulsants of the new generation: Gabapentin, Pregabalin (Lyrics). The use of these drugs can reduce the intensity of the pain syndrome due to their action on the central nervous system;
• Novocain intravenously during an attack with the aim of anesthesia;
• tranquilizers: Aminazine, Diazepam (Sibazon, Relanium). The use of this group of drugs is possible only in cases of severe pain syndrome, not removed by other analgesics.

From physiotherapeutic methods apply:

• baths (radon, sulphide);
• galvanization (areas of sympathetic nodules, a Shcherbaku collar);
• mud applications;
• darsonvalization;
• ultraviolet irradiation of the zone of sympathetic ganglia;
• acupuncture.

When the disease is particularly severe and seizures become very long, exhausting the patient, and the standard program of conservative drugs is powerless, resort to surgical treatment. The operations consist in excision of sympathetic nodes (sympathectomy), responsible for innervation of the affected area. After such operations, the vessels remain without control from the nervous system, and the flow of blood is not accompanied by the onset of pain.

Preventive measures in relation to the occurrence of seizures is the recommendation to avoid overheating, to wear loose shoes. It is necessary to exclude smoking and the use of alcoholic beverages, as this provokes occurrence of an attack of erythromelalgia.

In conclusion, I would like to say that erythromelalgia is not at all dangerous to life, but it affects its quality very much. Completely get rid of this disease, for today, it is impossible. However, it is quite possible to reduce the number of seizures to a minimum. Patients with erythromelalgia should not despair. With all the recommendations of the attending physician, the victory will be on the patient's side!