Melkersson-Rosenthal syndrome

In which Melkersson-Rosenthal syndrome it is an inflammatory disease. The disease belongs to the category of so-called orofacial granulomatoses. The Melkersson-Rosenthal syndrome is usually characterized by a combination of three typical symptoms. These complaints are, on the one hand, swelling of the lips, on the other hand, so-called wrinkled tongue, and finally, peripheral facial paresis.

What is Melkersson-Rosenthal Syndrome?

The Melkersson-Rosenthal syndrome occurs in the majority of cases in young adult patients. It is also true that the disease occurs more frequently in women than in men.

Basically, the syndrome is an idiopathic inflammatory disease. The disease was named after two doctors, namely Ernst Melkersson and Curt Rosenthal. The Melkersson-Rosenthal syndrome is principally characterized by the common occurrence of three essential symptoms.

causes

In principle, the exact causes for the development of Melkersson-Rosenthal syndrome have not yet been fully clarified according to the current state of medical knowledge. In principle, the disease is a so-called granulomatous inflammatory disease.

In some cases, affected patients are linked to intolerance to various foods. In addition, Melkersson-Rosenthal syndrome may also occur in people who have Crohn's disease. The same applies to patients with sarcoid. The Melkersson-Rosenthal syndrome is based on a granulomatous inflammation.

Symptoms, ailments & signs

In principle, Melkersson-Rosenthal syndrome is a relatively rare disease. It is counted among the granulomatous inflammations. In numerous cases, Melkersson-Rosenthal syndrome begins in adolescence or early adulthood.

The disease most commonly affects people between the ages of 20 and 40. The main symptoms of Melkersson-Rosenthal syndrome are granulomatous inflammatory processes and edematous swollen lips. In the majority of cases, the upper lip is affected by the typical swelling.

The swelling occurs much less often on both lips or only on the lower lip. In addition, the patient's palate or cheek area may also be affected. Sometimes changes occur on the tongue, which then resembles a map in its appearance.

It is also possible that the tongue will enlarge. In addition, in some cases paralysis of the facial nerves can be seen. In some cases, however, these only appear months or even years after the swollen lips. Some patients develop neurological symptoms such as meningitis or encephalitis.

The peripheral paralysis of the facial nerve takes the form of a sudden attack. Periods without any symptoms are also possible, which are followed by intervals with complaints. The swelling of the lips is also known as granulomatous cheilitis in the context of Melkersson-Rosenthal syndrome. The swollen lips can be pressed in.

If the swelling persists for a long time, a fissure may form. The third typical symptom of Melkersson-Rosenthal syndrome, the wrinkled tongue, is also called the lingua plicata. Deep furrows appear on the surface of the tongue, and fissures sometimes form.

In addition, many patients have ulcers on the mucous membrane in the mouth. These may have a pronounced rim wall, but in other cases only appear as superficial canker sores. These ulcers are often accompanied by swelling or reddening of the oral mucosa.

In addition, swollen lymph nodes in the neck area can be felt. Basically, the course and prognosis of Melkersson-Rosenthal syndrome are difficult to assess. In some cases there are spontaneous remissions, and a protracted course of the disease is also possible.

Some patients also suffer from relapses. As a rule, the Melkersson-Rosenthal syndrome is characterized by an intermittent course, with the swollen lips usually receding. During the illness, the tissue that is no longer capable of regression may increase.

Diagnosis & course of disease

The diagnosis of Melkersson-Rosenthal syndrome is based on various research methods. The typical clinical appearance of the disease easily leads to a suspected diagnosis, which is confirmed with the help of further measures. To reliably diagnose Melkersson-Rosenthal syndrome, for example, biopsies of the skin or mucous membrane and laboratory diagnostics are possible.

Among other things, the C-reactive protein is determined in the blood. It is important to rule out Crohn's disease and sarcoid as part of the differential diagnosis. X-rays and a colonoscopy are usually used for this purpose.

Complications

The Melkersson-Rosenthal syndrome primarily causes swelling and therefore paralysis of the face. The lips and tongue in particular are swollen and various disorders of sensitivity occur throughout the face. The quality of life of the patient is considerably reduced and restricted by these swellings. In many cases, those affected are dependent on the help of other people in their everyday lives.

In particular, the intake of food and liquids can be affected by Melkersson-Rosenthal syndrome. Limitations in speaking can also occur. As a rule, self-healing does not occur, so that those affected are dependent on medical treatment. Furthermore, the symptoms come on very suddenly, so that it is not uncommon for psychological upset or severe depression to occur.

The symptoms of Melkersson-Rosenthal syndrome can be limited with the help of medication. However, a positive course of the disease cannot be guaranteed in every case. In some cases, the paralysis cannot be completely resolved, so those affected have to live with various limitations. Life expectancy itself is usually not influenced by Melkersson-Rosenthal syndrome.

When should you go to the doctor?

Optical changes in the lips are a sign of impaired health. A doctor's visit is necessary as soon as there is repeated or persistent lip swelling. If the person concerned suffers from inflammation, internal irritation or a slightly increased body temperature, the complaints should be clarified. Lips sensibility disorders, numbness or hypersensitivity should be examined and treated. If food is refused or if there is an unwanted weight loss, the person concerned needs medical help. If there are additional emotional problems or mental irregularities due to the visual abnormalities, a visit to the doctor is advisable.

In the event of social withdrawal, fluctuations in mood or depressive phases or other behavioral problems, a check-up visit to a doctor is recommended. A doctor is required if the oral mucous membrane is reddened, canker sores or other changes in the appearance of the skin in the mouth. Pain, regression of the gums, or bleeding in the mouth indicate a condition that should be diagnosed and treated. In many cases there is spontaneous healing. Nevertheless, a doctor's visit should be made, as the symptoms will most likely recur after a few weeks or months. A doctor will be needed in the event of swollen lymph nodes, palpable lumps on the neck or general malaise.

Treatment & Therapy

There is currently no causal therapy for Melkersson-Rosenthal syndrome. Usually steroids like cortisone are used. Glucocorticoids or NSAIDs are given to relieve symptoms. Immunosuppression using clofazimin, azathioprine and thalidomide is also possible.

Cortisone is used for mild swelling, while glucocorticoid is injected for more severe swelling. In principle, the symptoms that occur in the context of Melkersson-Rosenthal syndrome are only treated symptomatically. The main aim of the efforts is to maintain and improve the quality of life of the affected patient despite the symptoms.

Outlook & forecast

The Melkersson-Rosenthal syndrome is now mostly referred to as orofacial granulomatosis. In most cases, the Melkersson-Rosenthal syndrome is episodic with inflammatory components. This course of the disease can become chronic. It can extend over years, often a whole life. In this case, there can be no optimistic forecast.

It may be consoling that most of those affected do not have the full picture of Melkersson-Rosenthal syndrome, but "only" minus variants with different symptoms and individual characteristics. The full picture of the Melkersson-Rosenthal syndrome is rarely found in children in particular.

Since the doctors have not yet been able to find the cause of Melkersson-Rosenthal syndrome, the disease may be due to a genetic defect. A familial accumulation speaks for this. After all, doctors nowadays know that Melkersson-Rosenthal syndrome can lead to spontaneous remissions. The disease has so far been viewed as a recurrent chronic disease. Accordingly, a cure would not be possible, but the absence of the inflammatory symptoms would be.

Since the course is individual for each person, it is difficult to predict. This also makes it difficult to make an accurate forecast. Life expectancy is usually not restricted with Melkersson-Rosenthal syndrome. The quality of life, however, depends on the severity of the symptoms. It is to be hoped that the proof of the cause and a gene therapy intervention will bring relief to the sick in the future.

prevention

Effective measures to prevent Melkersson-Rosenthal syndrome are currently unknown. Because the causes for the development of the disease are still not sufficiently researched. The patient's cooperation is essential to alleviate the symptoms.

Aftercare

In most cases, Melkersson-Rosenthal syndrome leads to severe swelling in the patient, which occurs primarily on the face. These swellings also significantly reduce the aesthetics of the person affected, so that most patients also suffer from reduced self-esteem or from depression and other psychological disorders. This can potentially lead to bullying or teasing children.

It is not uncommon for the Melkersson-Rosenthal syndrome to make it much more difficult to take food and fluids, so that the person affected suffers from various deficiency symptoms and from being underweight. Furthermore, the syndrome also leads to breathing difficulties, so that the patient's resilience is significantly reduced by this disease.

Most of those affected cannot actively participate in everyday life and also suffer from restrictions in their movement. The swelling of the tongue makes it difficult to speak, which can lead to slower development in children. Self-healing cannot occur in Melkersson-Rosenthal syndrome, and the general course cannot be predicted either. The disease may lead to a reduced life expectancy for the patient.

You can do that yourself

Melkersson-Rosenthal syndrome can only be treated symptomatically. That is why the most effective self-help measure is to have the individual symptoms and complaints clarified and treated at an early stage. In addition to drug therapy, those affected can take other steps to alleviate the symptoms and improve their quality of life.

Physical activity is particularly recommended. Regular physical exercise improves well-being and individual inflammatory processes are slowed down. A balanced and healthy diet has a similar effect. Those affected should work with their doctor or a nutritionist to create a nutrition plan that is tailored to the individual symptoms and complaints. In principle, foods that trigger or promote inflammatory processes should be avoided. These include, for example, alcohol and ready-made meals, but also certain types of vegetables and fruits. The attending physician can best answer which food and drinks are allowed.

Finally, it is important to avoid stress and to protect the body. If medical therapy is carried out at the same time, the progression of the disease can at least be slowed down. To avoid complications, the course of Melkersson-Rosenthal syndrome should be monitored by a doctor.