Melkersson – Rosenthal syndrome is an inflammatory disease. The disease belongs to the category of so-called orofacial granulomatosis. 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 a so-called wrinkled tongue and finally a peripheral facial nerve paresis.
What is Melkersson-Rosenthal Syndrome?
Melkersson-Rosenthal syndrome occurs in the majority of cases in young adult patients. It is also true that the disease occurs with a greater frequency in women than in men. See AbbreviationFinder for abbreviations related to Melkersson-Rosenthal Syndrome.
Basically, the syndrome is an idiopathic inflammatory disease. The condition was named after two doctors, namely Ernst Melkersson and Curt Rosenthal. The Melkersson-Rosenthal syndrome is principally characterized by the joint occurrence of three essential symptoms.
Basically, the exact causes for the development of the 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 show a connection 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 sarcoidosis. 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 many 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.
It is much less common for the swelling to appear on both lips or only on the lower lip. In addition, the palate or the cheek area of the diseased patient 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 enlarges. In some cases, there is also paralysis of the facial nerves in the face. However, these sometimes only appear months or even years after the swollen lips. Some patients develop neurological symptoms such as meningitis or encephalitis.
Peripheral facial nerve palsy occurs in the form of a sudden seizure. It is also possible to have periods without any symptoms, which are replaced by intervals with symptoms. The swelling of the lips is also referred to as granulomatous cheilitis in the context of Melkersson-Rosenthal syndrome. The swollen lips can be pressed in.
If the swelling persists for a long period of time, a fissure may form. The third typical symptom of Melkersson-Rosenthal syndrome, the folds of the tongue, is also called lingua plicata. Deep furrows appear on the surface of the tongue, and sometimes fissures also form.
In addition, numerous patients develop ulcers on the mucous membrane in the mouth. These may have a pronounced edge wall, but in other cases they only appear as superficial aphthae. 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 is spontaneous remission, and a protracted course of the disease is also possible.
Some patients also suffer from recurrences. As a rule, Melkersson-Rosenthal syndrome is characterized by an intermittent course, with the swollen lips usually regressing. During the disease, the tissue may increase, which is no longer capable of regression.
Diagnosis & disease progression
The diagnosis of Melkersson-Rosenthal syndrome is based on various examination methods. The typical clinical appearance of the disease easily leads to a suspected diagnosis, which is substantiated with the help of further measures. In order to diagnose Melkersson-Rosenthal syndrome with certainty, 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 sarcoidosis as part of the differential diagnosis. X- rays and colonoscopy are usually used for this purpose.
Melkersson-Rosenthal syndrome primarily causes swelling and thus also paralysis in the face. The lips and tongue in particular are swollen and various sensory disorders occur throughout the face. The patient’s quality of life is significantly reduced and limited by these swellings. In many cases, those affected are dependent on the help of other people in their everyday lives.
Especially the intake of food and liquids can be affected by Melkersson-Rosenthal syndrome. Speaking limitations can also occur. As a rule, self-healing does not occur, so that those affected are dependent on medical treatment. Furthermore, the symptoms occur very suddenly, so that it is not uncommon for mental disorders 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 that those affected have to live with various restrictions. Life expectancy itself is usually not affected by Melkersson-Rosenthal syndrome.
When should you go to the doctor?
Optical changes in the lips are a sign of a health impairment. A doctor’s visit is necessary if there is repeated or persistent swelling of the lips. If the affected person suffers from inflammation, internal irritation or a slightly elevated body temperature, the symptoms should be clarified. Sensitivity disorders of the lips, numbness or hypersensitivity should be examined and treated. If food is refused or if weight is lost unintentionally, the affected person needs medical help. If additional emotional problems or mental irregularities appear due to the visual abnormalities, a doctor’s visit is advisable.
In the case of social withdrawal, mood swings or depressive phases as well as other behavioral problems, a check-up with a doctor is recommended. A doctor is required in the event of reddening of the oral mucosa, aphthae or other changes in the appearance of the skin in the mouth. Pain, receding gums, or bleeding in the mouth indicate a condition that should be diagnosed and treated. In many cases, spontaneous healing occurs. Nevertheless, a doctor’s visit should be made, since the symptoms will most likely return after a few weeks or months. A doctor is needed for swollen lymph glands, the formation of 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 with clofazimine, azathioprine and thalidomide is also possible.
Cortisone is used for slight swelling, while glucocorticoid is injected for more severe swelling. In principle, the treatment of the complaints that occur as part of the Melkersson-Rosenthal syndrome is only symptomatic. The focus of the efforts is the goal of maintaining and improving the quality of life of the affected patients despite the symptoms.
Outlook & Forecast
Today, Melkersson-Rosenthal syndrome is usually referred to as orofacial granulomatosis. In most cases, when Melkersson-Rosenthal syndrome is present, there is an intermittent course with inflammatory components. This course of the disease can become chronic. It can last for years, often even a lifetime. In this case, there can be no optimistic forecast.
It may be comforting 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. Especially in children, the full picture of Melkersson-Rosenthal syndrome is rarely found.
Since doctors have not yet been able to find out the cause of Melkersson-Rosenthal syndrome, the disease may be due to a genetic defect. A familial accumulation speaks for this. After all, doctors now know that spontaneous remissions can occur in Melkersson-Rosenthal syndrome. The disease has so far been regarded as a relapsing chronic disease. Accordingly, a cure would not be possible, but the absence of the inflammatory symptoms would be.
Since the course is different for each person, it is difficult to predict. This also makes it difficult to make an accurate prognosis. Life expectancy is usually not limited in Melkersson-Rosenthal syndrome. The quality of life, however, very much depends on the severity of the symptoms present. It is to be hoped that the proof of the cause and a gene therapy intervention will bring relief to those affected in the future.
There are currently no known effective measures to prevent Melkersson-Rosenthal syndrome. Because the causes of the formation of the disease have not yet been sufficiently researched. Patient cooperation is key to alleviating symptoms.
In most cases, the Melkersson-Rosenthal syndrome causes severe swelling in the patient, which mainly occurs on the face. This swelling also significantly reduces the aesthetics of the affected person, so that most patients also suffer from reduced self-esteem or from depression and other psychological upsets. This can lead to bullying or teasing in children.
It is not uncommon for Melkersson-Rosenthal syndrome to make it significantly more difficult to take in food and liquids, so that the person affected suffers from various deficiency symptoms and is underweight. Furthermore, the syndrome also leads to breathing difficulties, so that the patient’s resilience is also 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 causes problems when speaking, which can lead to slower development in children. Self-healing cannot occur in Melkersson-Rosenthal syndrome, and the general course cannot be predicted. The patient’s life expectancy may be reduced as a result of the disease.
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 further steps to alleviate the symptoms and improve their quality of life.
Above all, physical activity is 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 draw up 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 meals, but also certain types of vegetables and fruit. The doctor treating you can best answer which foods 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.