Erythromelalgia - neurogenic vascular damage

Causes and mechanism of development of the disease

There are primary and secondary forms of the disease.
The first, acting as an independent disease, develops separately. Secondary occurs as a symptom of some other diseases:

• multiple sclerosis;
• myxedema;
• osteochondrosis;
• hypertension;
• consequences of injuries and frostbite;
• blood diseases.

Secondary erythromelalgia develops as one of the symptoms of another disease, on which its course and treatment depend.

The primary one arises on its own, and its causes have not yet been established; at this stage of the development of medicine, experts put forward several versions:

1. The first suggests that the disease is a consequence of peripheral neuritis .
2. The second is that the disease is of spinal origin , and develops as a result of a radical inversion of the gray matter of the spinal cord, resulting in weakening of the vasomotor fibers. This version is supported by the presence of structural changes in the spinal cord in a significant proportion of patients.
3. The third theory associates the disease with various malfunctions in the sympathetic nervous system or pathologies of the walls of blood vessels.
4. The fourth version speaks about the influence of the characteristics of the individual’s psyche . Thus, Mitchell's disease often occurs in children prone to psychosis.

So far, none of the versions has been recognized by the scientific community, and research is still ongoing.

During attacks, a large influx of blood enters the vessels of the arms and legs, which does not fit into the volume of the capillaries. In the vessels that are located in these places, there are a large number of receptors, and with a sharp expansion of the capillaries, the receptors are irritated, causing pain, redness and fever.

Main sources of occurrence

The etiological origin of erythromelalgia is still under investigation. However, doctors say that the basis of the disorder is an abnormality in the nervous regulation of the choroid. A consequence of the failure of the stable functioning of the vasomotor system is the formation of local dilatation in the peripheral part of small and medium-sized vessels. As a result, the patient experiences a restructuring of blood flow towards an increase in the influx of blood cells of an arterial nature. The patient's clinical history shows redness.

Vascular dilation is combined with an increased level of permeability of the cavities, which ultimately provokes the formation of swelling. According to tests, the formation of pain is caused by the release of humoral particles and overirritation of the sympathetic departments. To date, medical staff have not been able to determine the degree of damage to the nervous system due to which the disease is formed. In some cases, abnormal abnormalities occurring in the posterior spinal cord, peripheral nerve roots, and trunks have been reported. Primary erythromelalgia refers to an independent lesion. The secondary form can occur with impaired functioning of the spinal cord, blood diseases, alcohol abuse, endocrine defects, abnormal metabolism, and vibration abnormalities.

Features of symptoms and diagnosis

The main symptom of erythromelalgia is pain in the lower arms and legs. As a rule, it appears suddenly, in the form of a strong and burning attack. When heated, for example, in the heat, it intensifies, as well as during a vertical position of the body.

In this case, relief may occur from cooling or a horizontal position. Attacks can also appear on the ears or the entire face. The rest of the time the patient feels completely healthy.

Other symptoms of Mitchell syndrome are:

• swelling of the limbs;
• symmetry of the lesion;
• the appearance of urticaria rash;
• increased temperature at the affected area;
• impaired sweating in the extremities;
• redness of the feet and palms, impaired sensitivity.

At the beginning of the development of the disease, attacks of pain appear only in the evening and at night, but in the absence of treatment, they can last for a day, while the area of ​​spread gradually increases.

If the disease is advanced, it can lead to tissue death in the extremities, thickening of the fingers, clouding and brittleness of the nails, as well as thickening and atrophy of the outer layer of skin.

With secondary erythromelalgia, symptoms are less pronounced and tissue changes occur to a lesser extent.

Diagnosis of the disease is carried out on the basis of anamnesis and analysis of the clinical picture, which includes a number of signs:

• the presence of throbbing pain that lasts for several minutes or several days;
• duration of attacks and their intensity;
• time of attack in accordance with the day and period of the year, position of the limb, ambient temperature and other factors;
• does the pain subside after applying a cold compress, walking on snow or wet sand;
• the place of initial pain and its further spread;
• symmetry of the disease, manifested in development on both arms or legs;
• external changes in the form of swelling, excessive sweating, bluish skin and others;
• development of thickenings on the nails and peeling of the skin, the appearance of cracks on it.

When conducting diagnostics, the main argument in favor of Mitchell's disease is the inability to identify another cause for the appearance of these symptoms.

Treatment options

The patient is prescribed a comprehensive course of therapy using pharmacological medications and physiotherapeutic techniques. Due to the fact that with erythromelalgia the main problem is the dilation of blood vessels, during an attack, means are used to narrow them. However, the doctor should prescribe such substances with caution, since their use is associated with the possibility of arterial disorders. To get rid of edema, antihistamines are needed. NSAIDs are suitable for pain relief.

The complex treatment course includes drugs that increase the strength of the walls of blood vessels. During the interparoxysmal stage, it is recommended to undergo reflexology sessions, take mud, hand and foot baths, and visit radon and carbon dioxide sources. In case of severe and poorly controlled symptoms, surgical intervention is prescribed. The doctor performs a symptaecotomy - elimination of the sympathetic sections that provoke innervation of the affected area. The operation does not eliminate the presence of attacks, but it helps relieve pain.

Providing medical care

Erythromelalgia is treated by a specialist - a neurologist.

Therapy includes a whole range of means and methods, which includes medication, physiotherapy and surgery, and in the secondary form, elimination of the underlying disease.

It is worth noting that today the disease is not completely cured; the effect will only be temporary.

Drug treatment includes taking drugs of different directions of action:

• painkillers;
• antihistamines;
• vasoconstrictors;
• corticosteroids;
• vitamins;
• calcium salts.

For severe pain, novocaine blockades and corticosteroid injections are prescribed.

Physiotherapeutic methods make it possible to increase the nervous system’s regulation of the tone of the vascular walls. At the same time, they help reduce and even eliminate the symptoms of the disease.

The main physiotherapeutic means are baths and baths with chilled or even cold water lasting from 5 to 10 minutes; they can be simple or with various additives (radon, salt, aromatic).

Baths have a thermal, chemical and mechanical effect on the affected areas, slow down the heart rate, reduce pain, enhance metabolic processes, and inhibition processes in the cerebral cortex. It is recommended to use them daily for 10-14 days, depending on the type.

Hardening with water or air and reflexology are also used.

If traditional treatment methods do not bring relief, the doctor prescribes surgery: sympathectomy, during which part of the nerve is removed. The operation is performed under general anesthesia and can be endoscopic or open.

Treatment of erythromelalgia with traditional methods allows you to strengthen the walls of blood vessels, increase their tone and normalize blood pressure. An alcoholic tincture of hawthorn or a decoction of its berries with honey is used, used daily in a course of 10-14 days.

It is also recommended to use garlic oil, which is used in a course of 3 months, and blue iodine.

How is erythromelalgia diagnosed?

If a standard clinical history is present, the neurologist can easily give a correct conclusion to the patient. It is worth paying attention to differential diagnostics to exclude the presence of other similar anomalies. Detection of sympathalgia is considered the main distinguishing feature of Mitchell's disease from acroerythroses. The latter lesion appears in damp and cold climates, and the described disorder consistently reduces the level of damage in cool weather. As diagnostic procedures, doctors resort to the following methods:

• Thermography.
• X-ray examination of the foot.
• Doppler ultrasound of vascular sections.
• Capillaroscopy.
• Magnetic resonance scanning of the lumbar spine.
• Ultrasound examination of the nerve.
• Electroneuromyography.
• Based on the indications, computed tomography or MRI testing of the foot is prescribed.
• Collection of blood cells for biochemical analysis.
• Assessment of the level of cortisol and hormonal elements in the thyroid gland.

If endocrinological abnormalities are detected, the patient will need to visit an endocrinologist's office, and if vascular abnormalities are detected, a consultation with a phlebologist or surgeon is needed.

Preventive measures

Carrying out preventive measures is problematic because the exact causes of the disease are not known. In order to reduce possible risks, several rules should be followed:

• avoid places where the temperature is very high, this applies to visiting saunas and baths, as well as countries with a hot climate;
• get rid of bad habits;
• avoid work that causes muscle strain and increased blood circulation;
• Avoid injuries and frostbite to hands and feet.

Erythromelalgia is rare, but it is not yet possible to clearly define the risk group, so the best way to avoid this disease is to maintain a healthy lifestyle and be attentive to your body, allowing you to notice the first symptoms of an unusual disease.

Erythromelalgia: causes, signs, symptoms, treatment

• Main manifestations
• Course of the disease
• Diagnostics
• How to get rid

Erythromelalgia is a disease that should be classified as rare.

The disease was first described in 1943 as a sudden onset of pain in the feet and a feeling of heat. The basis of the disease is paroxysmal dilatation of blood vessels, or rather, small arteries, in the arms or legs, less often in the area of ​​the face, ears or knees.

At the same time, a severe attack of pain begins, the temperature of the skin increases and its redness occurs.

This disease can be primary or secondary. In the first case, the cause is unknown. In the second, the cause is diseases such as polycythemia, thrombocythemia, venous insufficiency, high blood pressure, diabetes mellitus, systemic lupus erythematosus, scleroderma, gout or multiple sclerosis.

Today, erythromelalgia can be independent or be a syndrome for a variety of primary diseases - neurological, somatic, or caused as a result of injuries, frostbite and overload of the body.

Main manifestations

Erythromelalgia has pronounced symptoms. Acute pain and redness of the skin of the legs or arms can last from minutes to several hours. Sometimes this phenomenon can be caused by minimal overheating of the body, for example, when exposed to temperatures from 29 to 32 degrees.

When you immerse your hand or foot in cold water, as a rule, all manifestations disappear quite quickly. Trophic disorders of the skin even with a long course of the disease do not occur. Symptoms may remain at the same level for many years without getting worse, but over time they still lead to disability.

Often all manifestations are accompanied by generalized vasomotor dysfunction, and Raynaud's phenomenon is also possible.

Primary erythromelalgia of the foot manifests itself in the form of a paroxysm of burning pain, which becomes especially severe in summer, in hot weather, and at night. At the very beginning, the pain appears only in the evenings and lasts until the morning.

Subsequently, its duration increases to a day. The big toe and heel are mainly affected, then the sole and dorsum of the foot are involved, and in the most severe cases even the lower leg.

The longer the period of the disease, the more affected areas.

The process is always two-way and symmetrical. But first one limb is affected, and only a little later – the second.

Course of the disease

The disease occurs in the form of painful attacks that can drive a person to despair. The affected area sharply and suddenly turns red, becomes hot, and may become covered in a rash. If the disease remains untreated for a long time, necrosis may develop.

In a horizontal position, the symptoms subside. The same thing happens when applying cold lotions. Outside of an attack, the patient feels absolutely healthy.

How to get rid

Treatment of erythromelalgia of the foot includes, first of all, the elimination of provoking factors. In the primary form, prostaglandin analogues may be effective. In the secondary form, the main treatment is aimed at the underlying pathology. Aspirin can be used.

Treatment should be comprehensive and eliminate all etiological factors. The basis of therapy is the use of vasoconstrictor drugs, which are used in both forms of the disease.

Also used in treatment are B vitamins, novocaine, physiotherapy, novocaine blockades, and acupuncture, which has proven to be one of the most effective.

It is important to wear only simple and comfortable shoes and avoid any overheating.

Only in the most severe cases, when symptoms cannot be treated, is surgery used. An operation called periarterial, preganglionic sympathectomy is used. For severe pain that does not go away even under the influence of painkillers, stereotactic surgery on the basal ganglia is used.

Symptoms

Many patients with thrombocythemia are asymptomatic. The first sign of a disorder may be the formation of a blood clot (thrombus). Although a blood clot can form in any part of the body, with thrombocythemia, blood clots most often occur in the brain, hands, and feet.

Signs and symptoms depend on the location of the blood clot. These include:

• Headache
• Dizziness or lightheadedness
• Chest pain
• Weakness
• Fainting
• Temporary visual impairment
• Numbness or tingling in the hands and feet
• Redness, throbbing, and burning pain in the hands and feet (erythromelalgia)
• Slightly enlarged spleen

In rare cases, bleeding may occur due to thrombocythemia, especially if the platelet count in the blood is very high (more than 1 million platelets per microliter of blood). Bleeding may manifest itself as follows:

• Nosebleed
• Bruising
• Bleeding in the mouth or gums
• Bloody stool

The clot can lead to a transient ischemic attack (TIA), in which blood supply to an area of ​​the brain is temporarily cut off, or to a stroke. Signs and symptoms appear suddenly and include the following:

• Weakness or numbness of the face, arm, or leg, usually on one side
• Slurred speech or difficulty understanding speech (aphasia)
• Blurred vision, double vision, or blurred vision

Conditions under which you need to see a doctor

If you experience signs and symptoms of abnormal blood clots or bleeding, consult your doctor.

If you experience signs and symptoms of a TIA or stroke, such as numbness or paralysis on one side of your body, you should contact your doctor right away.

Complications

The blood clotting abnormality of thrombocythemia can cause a number of potentially serious complications, such as:

Complications during pregnancy. Despite the fact that many women suffering from thrombocythemia have pregnancy without complications, it is better to be under the supervision of a doctor during this period.

Without proper treatment, thrombocythemia can cause miscarriage, premature birth, high blood pressure (preeclampsia), premature separation of the placenta from the uterine wall (placental abruption), and slow fetal development.

The risk of complications can be significantly reduced by regular examinations and taking appropriate medications. Stroke. A blood clot that blocks blood supply to the brain can cause a stroke. If you experience signs and symptoms of a stroke, seek emergency medical attention. Myocardial infarction.

A blood clot that blocks the blood supply to the heart can lead to a myocardial infarction (heart attack).

If you notice signs of myocardial infarction, such as tightness, a feeling of fullness or squeezing pain in the center of the chest that lasts more than a few minutes and also radiates to the shoulder, arm, back, teeth or jaw, shortness of breath, or sweating, you should seek medical help. emergency medical care. With essential thrombocythemia, bleeding (hemorrhage) may also occur, leading to significant blood loss. A small proportion of patients with essential thrombocythemia may subsequently develop acute leukemia or myelofibrosis. Both of these diseases are life-threatening.

Acute leukemia. Acute myelogenous leukemia is a rapidly progressing cancer of the blood and bone marrow. Myelofibrosis. This progressive bone marrow disorder causes scar tissue to form in the bone marrow, severe anemia, and an enlarged liver and spleen.

Causes

Erythromelalgia can be either a separate disease or a symptom of other diseases. The cause of the independent illness still remains unclear, because in most cases, except for severe and sudden pain in the feet, the person does not complain about anything else.

In all other cases, such pain may be a symptom of the following conditions:

1. Syringomyelia.
2. Multiple sclerosis.
3. Consequences of injuries.
4. Hypertonic disease.
5. Myxedema.
6. Overheat.
7. The result of a burn.

The primary form of the disease is rare, but the secondary form is much more common, especially with diseases such as diabetes mellitus, Raynaud's disease in the third stage, and endarteritis.

Pathogenesis

No one knows exactly why erythromelalgia of the foot occurs. The main cause is believed to be peripheral neuritis, which was treated surgically. After removing the affected nerve, impulses from the nerve endings that were affected were completely eliminated.

But other researchers believe that the cause of the disease is completely different. In their opinion, pain symptoms in the feet arise from changes in the gray matter located in the spinal cord. In this case, complete paralysis of the vasomotor fibers occurs.

To confirm this, it is worth saying that this pain symptom most often occurs precisely when the spine is damaged.

There is an opinion that erythromelalgia is a kind of neurosis and occurs in individuals with special features in the mental system. So it is quite difficult to understand exactly why the disease begins to develop, and the pathological data do not show anything - it is impossible to identify the disease after a person’s death.

How does the disease manifest itself?

This disease is characterized by the following series of symptoms:

1. Acute pain.
2. Increased foot temperature.
3. Redness of the feet.

In most patients, this condition occurs in the summer, and if the affected limb is immersed in ice water, the burning and unbearable pain begins to quickly pass. Trophic changes in tissue do not occur with this disease.

The pain can worsen not only in summer, but also at night, after sleeping in a warm bed. At the very beginning, pain occurs only in the evenings, but it can last all night. As it develops, the pain may not go away throughout the day. First, the big toe or heel begins to hurt.

Then the sole, the back of the foot, and in some cases even the lower leg are involved in the process. But not only the feet suffer from this disease. Sometimes the tip of the nose or even the earlobe may hurt.

At first, the phenomenon occurs only on one side, but gradually the second limb is also symmetrically affected.

Correct therapy

If a primary disease is identified, then it is better to begin treatment by eliminating thermal factors, resting and placing the legs in an elevated position. Among medications, gabapentin and its analogues can be used. But with secondary erythromelalgia, therapy should be aimed at the underlying disease, which caused severe pain in the legs.

Tools such as:

1. Vasoconstrictor drugs.
2. Vitamin B 12.
3. Histamine therapy.
4. Injection of novocaine or procaine into the affected area.
5. Physiotherapy.
6. Acupuncture.

If all else fails, then you have to resort to surgery.

If children are diagnosed with Raynaud's syndrome, this fact must be taken into account when choosing a future profession. In the future, they are strictly prohibited from hypothermia, nervous strain, and vibrations. Therefore, the disease requires some attention to the environment and daily routine.

Source: https://healthage.ru/poleznye-sovety/lechenie-boleznej/eritromelalgiya-prichiny-priznaki-simptomy-lechenie/