Melkersson-Rosenthal syndrome (MRS) is a rare neurological condition with three characteristic features. These include recurring facial paralysis, facial swelling, and furrows in the tongue.

MRS can affect people of all ages, but it typically starts during childhood or adolescence. To receive a diagnosis of MRS, a person will typically experience two or more of the classic symptoms. Currently, there is no cure for the condition, but treatment options may help reduce the severity of symptoms. A person may find working with a doctor helpful in managing MRS.

In this article, we will discuss what MRS is, including its possible causes, symptoms, and more.

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MRS, also known as orofacial granulomatosis, is a rare neurological condition. Three main features characterize the condition and include:

  • long lasting and recurrent swelling of the face, often in the upper lip
  • weakness in facial muscles
  • deep groves on the tongue

Typically, a person will need to experience at least two of the above criteria to receive a clinical diagnosis. The condition may flare intermittently and recur at intervals of days to months. MRS can affect anyone of any age, but it typically starts during childhood or teen years.

However, a person may only require one clinical feature to receive a diagnosis if there is evidence of granulomatous cheilitis after a biopsy. The term granulomatous cheilitis refers to lumpy swelling of the lips.

The condition affects an estimated 0.08% of the general population. It may affect more females than males, but this is not clear due to limited data.

There is currently no cure, but treatments can generally help. Though no standard of care is available, treatments can help with the symptoms by both reducing their frequency and decreasing their duration.

The condition can also affect people differently. Some may experience only mild symptoms, while others may experience more severe symptoms.

The exact cause of MRS is currently unknown. Some research suggests there may be genetic factors for the condition developing. However, as the presentation of symptoms is highly variable, this makes diagnosis and observing inheritance challenging. At present, scientists have not identified alterations in specific genes that may lead to MRS.

However, other research highlights that the following factors likely play a role in the development of MRS:

  • bacterial or viral infections
  • hormonal changes
  • dietary and other allergens

Additionally, research indicates that MRS is a granulomatous condition. This refers to when the immune system has difficulty fighting off certain pathogens and forms granulomas, which are clusters of white blood cells and other tissues, leading to inflammation. This is why it is important to rule out other granulomatous conditions, such as Crohn’s disease or sarcoidosis, during a diagnosis, as they can also present with oral symptoms.

There are three main symptoms that occur due to MRS. They include:

  • facial paralysis or muscle weakness
  • swelling of the face and often upper lip, which a doctor may refer to as orofacial edema
  • development of folds and furrows in the tongue

Health experts note the following about symptom occurrence:

  • about 8–18% of individuals will develop all three symptoms
  • roughly 30–80% will develop a fissured tongue
  • about 30–90% will develop facial weakness or paralysis, with roughly 10% experiencing recurrent paralysis
  • roughly 13–50% will develop both facial edema and paralysis in the face

While several symptoms typically occur with MRS, evidence notes that the condition may occur with nonspecific symptoms and overlap with alternative conditions. As such, diagnosis may often involve the need to rule out other conditions.

Other research notes that diagnosis may be possible if there is a combination of persistent or recurrent facial swelling and tongue fissures or facial paralysis. However, they also note that similarities with other conditions may mean diagnosis takes several years.

A doctor may take a biopsy or small sample of tissue from affected areas. This can help them rule out other conditions, such as:

  • sarcoidosis
  • cancer
  • recurrent angioedema
  • Crohn’s disease

Treatment typically depends on the presenting symptoms. In some cases, the symptoms may resolve without medical intervention. However, not treating the condition may lead to longer and more frequent recurrence.

A doctor may recommend the use of nonsteroidal anti-inflammatory drugs, such as ibuprofen, and corticosteroids to help with swelling. They may also suggest other medications, such as antibiotics to help fight infections or immunosuppressants to prevent the immune system from overreacting.

In some cases, a doctor may also recommend surgery. This may help relieve pressure on facial nerves and help reduce swollen tissue. They may also suggest other options, such as massage and electrical stimulation. However, more research into these treatment options is necessary.

MRS can recur intermittently throughout a person’s life and become a chronic condition. Medical interventions may help reduce the recurrence and severity of symptoms. If a person has other symptoms, they may want to work with a doctor to help rule out other potential causes. Treating the correct condition can help improve a person’s outlook.

A person may find that their symptoms improve on their own. They may only need minimal intervention to help improve their symptoms. Others may need more extensive care, which can include surgery. If someone needs surgery, they will need some recovery time following the procedure.

MRS is a rare neurological condition. It typically causes a person to experience two or more out of three characteristic symptoms. These symptoms include facial swelling, facial paralysis, or fissures on the tongue.

There is currently no cure for the condition. However, treatment can help shorten the duration of symptoms and potentially slow recurrence rates. Treatments typically involve medication therapies to reduce swelling. In some cases, a person may also require surgery.

MRS is a lifelong condition. A person can develop symptoms intermittently throughout their life. In some cases, treatment may not be necessary for symptoms to clear, but it can help clear them faster.