Melkersson-Rosenthal Syndrome (MRS)

What is Melkersson-Rosenthal syndrome?

Melkersson-Rosenthal syndrome (MRS) is a rare neurological disorder that affects facial nerves. It can cause facial swelling, furrows (grooves) in your tongue and facial paralysis.

MRS may signify a future diagnosis of Crohn’s disease or sarcoidosis. The condition is also called orofacial granulomatosis.

Who might get Melkersson-Rosenthal syndrome?

MRS can affect anyone at any age. But symptoms usually start in young adulthood.

How common is Melkersson-Rosenthal syndrome?

MRS is very rare. It affects about 0.08% of the population. But scientists believe it’s underdiagnosed because the symptoms can be similar to other conditions.

What causes Melkersson-Rosenthal syndrome?

Scientists don’t know what causes Melkersson-Rosenthal syndrome, but it’s been associated with:

• Crohn’s disease.
• Genetic inheritance.
• Hypersensitivity reaction (the body’s exaggerated immune response to an antigen or allergen).
• Sarcoidosis.
• Viral or bacterial infection.

Is Melkersson-Rosenthal syndrome an autoimmune disease?

The condition isn’t considered an autoimmune disease (when your immune system mistakenly attacks and destroys healthy body tissues).

MRS is classified as a neurological disorder and a granulomatous disorder. A neurological condition involves dysfunction in part of your brain or nervous system. A granulomatous condition involves one or more masses of tissue that get chronically inflamed.

What are the symptoms of orofacial granulomatosis?

The three classic Melkersson-Rosenthal syndrome symptoms are:

• Facial palsy: Facial weakness or paralysis may occur on one side or both. It may not happen until later episodes. Paralysis may last longer with each episode as the disease progresses.
• Facial swelling: People with Melkersson-Rosenthal syndrome usually experience swelling of their face (orofacial edema), lips (cheilitis granulomatosis) or both. Their upper lip swells before their lower lip. Lips may become hard, cracked, painful and reddish-brown. Other areas that may swell include cheeks, eyelids and scalp. Swelling may worsen and last longer with each episode.
• Furrowed tongue: The top of your tongue may develop deep grooves, also known as scrotal tongue or lingua plicata. This symptom usually doesn’t hurt, but it can cause swelling, itching and burning. It also may lead to loss of taste buds or oral infections.

Some people with MRS experience one or two of the classic symptoms. Others have all three.

The symptoms often flare up intermittently and repeatedly. They may last only a few hours the first time. But they tend to last longer and be more severe in subsequent episodes.

Other symptoms of MRS may include:

• Dizziness.
• Migraine headaches.
• Tinnitus or hearing loss.
• Scars from repeat, severe flare-ups.
• Trouble swallowing.
• Vision problems, such as dry eyes, blurred vision or uveitis (eye inflammation).

How is Melkersson-Rosenthal syndrome diagnosed?

MRS diagnosis is often difficult because the symptoms can be confused with other conditions. Examples include allergic reactions and Bell’s palsy.

Your healthcare provider usually diagnoses MRS based on a history of at least two of the classic symptoms. They may perform a biopsy on a tissue sample from your lips to confirm the diagnosis or rule out other conditions.

You may be referred to other specialists, such as:

• Allergist.
• Dermatologist (skin specialist).
• Gastroenterologist (digestive disorders specialist).
• Immunologist (immune system specialist).
• Ophthalmologist (eye and vision specialist).

Is there a cure for Melkersson-Rosenthal syndrome?

There isn’t a cure for MRS. The symptoms may resolve without treatment but recur (come back) in future flare-ups.

What is the treatment for Melkersson-Rosenthal syndrome?

If symptoms recur and are bothersome, certain treatments can lessen pain, prevent infections and improve appearance, such as:

• Antibiotics.
• Antihistamines.
• Corticosteroid injections.
• Immunosuppressants.
• Methotrexate.
• Nonsteroidal anti-inflammatory drugs.
• Surgery to reduce swollen lips or relieve pressure on facial nerves.
• TNF-alpha inhibitors, which help stop inflammation.

How can I prevent Melkersson-Rosenthal syndrome?

Scientists don’t understand the causes of MRS, so there aren’t any proven strategies to prevent it.

Does Melkersson-Rosenthal syndrome go away?

The outlook for people with MRS varies widely. Some people have mild flare-ups every few years that eventually stop on their own. Others have frequent flare-ups every few days that get worse each time.

The condition can be chronic for life, and swelling can become permanent. But scientists don’t think the disorder has any effect on life span.

What else should I ask my doctor about MRS?

If you have Melkersson-Rosenthal syndrome, consider asking your healthcare provider the following questions:

• How are you sure that this is Melkersson-Rosenthal syndrome?
• What other specialists should I see?
• When should I seek medical attention for a flare-up?
• Should I be tested for Crohn’s disease or sarcoidosis?
• How can I prevent infections, permanent swelling and scars?
• Would you recommend genetic testing?
• Do you know of any support groups for people with this condition?

A note from Cleveland Clinic

Melkersson-Rosenthal syndrome (MRS) is a rare neurological disorder that affects your facial nerves. If you experience episodes of facial swelling, facial paralysis or grooves on your tongue, talk to your healthcare provider. Treatments can help reduce symptoms and prevent complications.