Rossolimo-Melkersson-Rosenthal syndrome: what is it, symptoms, treatment of the disease

Melkersson-Rosenthal syndrome (there are the names of Mischer-Melkersson-Rosenatl, as well as Rossolimo-Melkersson-Rosenthal) is a rare disease characterized by a group of symptoms of chronic recurrent character, which are expressed in swelling of the lips, neuritis of the facial nerve, the formation of folds in the tongue.

Among the provoking factors are the development of an infectious disease, genetic prerequisites, and an allergic reaction. Treatment consists in taking medications, performing physiotherapy, and sometimes in surgery.

Causes of Melkersson-Rosenthal syndrome

The syndrome is more often diagnosed in the fairer sex, and at any age. If treatment is not started on time and the source of the disease is not eliminated, unpleasant symptoms will bother a person throughout his life, with an increase in frequency and frequency.

In modern medicine, there is not enough information about the causes of the syndrome. There are suggestions that clinical manifestations develop due to a malfunction in the activity of the hypothalamus.

There are reasons for this:

• a history of head injury;
• general intoxication;
• overheating of the body, including that caused by sunstroke;
• hormonal disbalance;
• endocrine disease;
• infectious brain damage.

Other sources of Melkersson syndrome include tonsillitis and sinusitis (other pathologies of similar localization), an inflammatory process in the lymph nodes located in submandibular space.

Often, symptoms occur on the background of an allergic reaction to a specific irritant (dust, pollen, pet hair, etc.). An equally significant role is played by a hereditary predisposition: if a close relative has such a disease, the risk of its development in offspring also increases.

The main symptoms

Melkersson-Rosenthal disease is characterized by a triad of symptoms - macrocheilitis (swelling of the lips), folding of the tongue, and paralysis of the facial nerve. Clinical manifestations occur suddenly, without precursor symptoms.

Swelling of the lip (or both lips) is the first sign of pathology. Less often, the initial manifestation is pain in the face, similar to neuralgia, which precedes the development of paralysis. In just 2-3 hours, there is swelling of the lips, an itchy syndrome, and other parts of the face are also involved in the lesion.

The lip becomes shapeless, begins to shine, but the color shade does not change. Rarely, the inflamed area becomes bluish. There is a proboscis-like eversion of the lip edge, it separates from the teeth, making them noticeable.

Swelling can be significant, with lips increasing 3-4 times when compared to natural size. There is uneven thickening: the left or right side is larger than the other. The edema has a soft or tight-elastic consistency. The imprint after pressing on the swelling does not remain.

Lip enlargement is present on an ongoing basis, growing and weakening from time to time. Sometimes the swelling suddenly disappears, subsequently occurring with greater intensity and frequency. Localization sites are not only the lips, but also the cheek (granulomatous pareitis), the eyelid, and other facial areas.

The second clinical manifestation is facial nerve palsy. Unilateral vasomotor rhinopathy occurs, unpleasant smack in the oral cavity, impaired production of salivary fluid. Due to a decrease in the tone of the muscles of the face, the corners of the mouth are lowered, the palpebral fissure widens. In most cases, incomplete paralysis is diagnosed, and sensitive, autonomic, motor functions are preserved.

The third clinical manifestation is folding of the tongue, which occurs in 80-85% of cases (Clinical protocols of the Ministry of Health of the Republic of Kazakhstan - 2016). The organ swells, its uneven increase occurs.

The syndrome does not always occur with a triad of symptoms. In some cases, only one or two clinical manifestations occur, for example, only macrocheilitis. Since the disease is chronic, it proceeds with stages of exacerbation and stages of remission. The syndrome is aggravated after an infectious pathology or against the background of emotional overstrain.

Diagnostics

First of all, the doctor asks about the disturbing symptoms. With Melkersson-Rosenthal syndrome, it is swelling on the lips, impaired speech, facial expressions, difficulty eating. Find out whether a person has a history of infectious pathologies, what is the level of genetic predisposition, whether there have been cases of hypothermia or, conversely, overheating of the body. Determine the characteristics of mental health.

In most cases, a preliminary diagnosis can be made already during the first examination of the affected areas. To confirm it and identify concomitant diseases, laboratory tests (blood tests, allergological tests) are prescribed.

They also carry out differential diagnosis of Melkersson-Rosenthal disease with other pathologies that are similar in clinical manifestations.

Differentiation table with other diseases:

Disease	Typical symptoms	Difference from Melkersson syndrome
Quincke's edema	The rapid progression of swelling of the tissues of the face, as well as the cervical spine, tongue, larynx.	Folding of the tongue and paresis of the facial nerve are not observed.
Lymphangioma	Slow development of puffiness (sometimes - up to several years).	They do not diagnose paresis of the facial nerve, folding of the tongue.
Hemangioma	An increase in the tissues of the lip or tongue is caused by the development of a benign tumor formed from blood vessels. It is characterized by slow development.	There is no folding of the tongue and paralysis of the facial nerve.
Lip abscess	Pain syndrome in the affected area, which increases with palpation.	They do not diagnose folding of the tongue, paresis of the face. The overall temperature rises.

After the diagnosis is confirmed, appropriate therapy is prescribed.

Pathology treatment

Treatment is not always effective. The drugs and physiotherapeutic procedures used are effective only at the initial stage of the development of the disease. In some cases, surgery is prescribed.

The complex eliminates concomitant pathologies, for example, chronic infectious foci, diseases of the central nervous system, heart, blood vessels, thyroid gland, and digestive tract.

Conservative

To reduce the intensity of puffiness, glucocorticoids are prescribed in combination with antibacterial drugs with a wide spectrum of action. From hormonal agents, preference is given to Prednisolone.

Of other medicines, the following are used:

• desensitizers (Suprastin, Tavegil);
• medicines with calcium (calcium gluconate);
• multivitamins and vitamin products (Supradin, retinol, nicotinic acid).

To correct the emotional state, sedatives and tranquilizers are prescribed. It is prohibited to accept such funds on your own.

To reduce the intensity of external manifestations, use Heparin ointment and Dimexide to apply applications. Rinse the mouth with chamomile infusion or a decoction of string and calendula. Compresses with hormonal ointments or Methyluracil can be performed.

From physiotherapeutic procedures, electrophoresis with drugs (for example, with heparin), ultrasound therapy, Bernard currents are used. Lidocaine, Trimecaine, Novocaine are used warm for blockade.

Conservative treatment lasts 1-1.5 months. If necessary, it is repeated every 2-3 months.

Surgical

Surgical intervention is prescribed if the conservative treatment carried out did not bring an improvement in the state of health and the edema did not decrease. During the operation, the doctor excises the tissue of the lip. The treatment does not help to achieve a stable remission and gives only a temporary result, allowing to hide the cosmetic defect.

Consequences and prognosis

Melkersson's disease is progressive and rapidly chronic. A person has a complex about his appearance, because extensive edema distorts the contours of the face and disfigures it. Frequent depressive conditions and mental disorders occur.

Due to the enlargement of the lips, their low mobility, difficulties with chewing food and swallowing it are observed, speech is distorted. In the cracks that appear on the tongue and lips, pathogens penetrate, which cause the development of infectious processes.

The risk of penetration of pathogens increases against the background of trauma to the mucous membranes by the teeth while chewing food. Therefore, patients with pathology need to make a diet so that only liquid food is present in it (grated, in a puree state). Fruits and vegetables are finely chopped before eating.

The prognosis for the disease is often favorable. The situation becomes more complicated if the larynx, nasopharynx, trachea are involved in the pathological process. The syndrome is aggravated if angiodystonic disorders occur, occurring with increased vascular permeability of the brain, edema of its tissues.

There is no specific prevention of the development of the syndrome in modern medicine. General recommendations include maintaining a healthy lifestyle, excluding every bad habit (smoking, drinking alcoholic beverages, taking drugs), timely treatment of provoking diseases.

If you manage to find symptoms similar to the manifestations of the syndrome, you should not self-medicate, but you need to see a doctor. The sooner therapeutic measures are taken, the more favorable the prognosis.