Orofacial granulomatosis is a rare skin condition that causes swelling in the face, mouth, or lips. The cause is often unknown, but experts think it’s related to allergies or an immune reaction.
“Orofacial” refers to conditions that affect your mouth and face. “Granulomatosis” refers to the formation of granulomas, a cluster of macrophages, which is a type of immune white blood cell.
Orofacial granulomatosis most often starts as swelling of your lips but may involve other parts of your face as well, such as your:
- inner lips
- gums
- the floor of your mouth
Many people who develop orofacial granulomatosis also develop Crohn’s disease, sometimes years after the symptoms of orofacial granulomatosis first appear.
Read on to learn more about orofacial granulomatosis, including causes, symptoms, and treatment options.
The underlying cause of orofacial granulomatosis usually isn’t known. Experts think it might relate to an immune reaction or an allergy to certain foods or food additives. A genetic predisposition and infections have also been posed as contributing causes.
Orofacial granulomatosis can occur by itself or with other inflammatory diseases like Crohn’s disease. Researchers think that orofacial granulomatosis with Crohn’s disease may represent a sub-type of Crohn’s disease with more extensive inflammation.
There remains debate regarding whether orofacial granulomatosis is a unique disease from oral Crohn’s disease. Crohn’s disease occurs in about 20% to 50% of people with orofacial granulomatosis.
Some evidence suggests that orofacial granulomatosis is caused by genes inherited from your parents.
The most common symptom of orofacial granulomatosis is swelling in your:
- lips
- mouth
- face
Swollen lips might crack in the middle or the corners of your mouth. Your gums might be painful and bleed while you brush your teeth. The pain may be made worse by eating food that’s:
- hot
- salty
- spicy
- hard
- abrasive
You may have open sores called ulcers. When these are present, they can last days to months. Swelling might persist for weeks to months.
Forms of orofacial granulomatosis
Orofacial granulomatosis has the following two variant forms:
- Melkersson-Rosenthal syndrome (MRS): MRS involves signs and symptoms like:
- facial nerve paresis on one side
- deep grooves in the tongue (lingua plicata)
- chronic lip and facial swelling on one side
- Miescher’s granulomatous cheilitis: This variant involves inflammation that only affects the lips.
Orofacial granulomatosis is often related to Crohn’s disease. Crohn’s disease typically involves complications like:
- anemia, where your body produces fewer blood cells than usual
- bone problems
- problems with growth and development in children
- malnutrition
- intestinal obstruction
- fistulas, atypical passages between two organs
- abscess, painful and pus-filled pockets
- anal fissures
- ulcers
It’s important to seek medical attention if you develop swelling around your lips and mouth that could be caused by orofacial granulomatosis. A doctor can test you for food allergies that may be causing your symptoms.
It’s also important to seek medical attention if you believe you may have Crohn’s disease. Symptoms of Crohn’s disease can mimic those of many other conditions but may include:
A doctor specializing in skin is called a dermatologist. They help diagnose orofacial granulomatosis. To diagnose, a dermatologist must take a sample of swollen tissue for laboratory testing.
Many conditions can cause swelling in your lips, so your doctor may want to order different tests to rule out other conditions. Some of the tests you might receive include:
- blood tests
- X-ray
- colonoscopy for Crohn’s disease
- patch tests for allergies
- tests for tuberculosis, as the symptoms of this condition can mimic orofacial granulomatosis
Currently, no cure for orofacial granulomatosis is available, but you can manage it with lifestyle changes and medications. Treatment options include:
- changes to your diet like avoiding cinnamon and benzoates, which are preservatives used to keep mold from growing
- topical corticosteroids in the forms of mouthwashes, inhalers, ointments and creams
- a short course of oral corticosteroids
- antiseptic alcohol-free mouthwash, spray, or gel
- topical immunosuppressant tacrolimus (Prograf, others) to treat lip swelling
If your doctor suspects a food allergy might be contributing to your symptoms, they may recommend an elimination diet. An elimination diet involves removing foods from your diet systematically to find the triggering food. About half of people experience an improvement with an elimination diet.
Additionally, treatment for Crohn’s disease might include:
- corticosteroids
- immunosuppressants
- biologics
- bowel rest
- surgery
There’s no cure for orofacial granulomatosis, but many people notice relief in their symptoms with corticosteroids or other treatments. Many people also notice relief in their symptoms after removing cinnamon and benzoates from their diet.
The cause of orofacial granulomatosis often isn’t clear, and it’s also unclear how to prevent it. For people with a known food allergy, avoiding this food might help prevent orofacial granulomatosis.
Is orofacial granulomatosis an autoimmune disease?
Researchers think that an immune reaction potentially plays a role in the development of orofacial granulomatosis, although the exact cause isn’t known.
What foods should you avoid with orofacial granulomatosis?
Many people notice relief in their symptoms when they remove cinnamon and benzoates from their diet. You can avoid or limit these foods and see if you notice any relief.
Does orofacial granulomatosis go away?
Symptoms of orofacial granulomatosis rarely go away without treatment, even when you remove a triggering food from your diet. Many people typically notice relief with treatments like steroids.
Is orofacial granulomatosis the same as Crohn’s disease?
Orofacial granulomatosis often occurs in people with Crohn’s disease. There’s still debate on whether it’s a separate condition or a form of oral Crohn’s disease.
Orofacial granulomatosis is a rare skin condition that causes swelling in your lips and around your mouth. The cause often isn’t known, but healthcare professionals suggest that it may relate to an immune reaction or allergy.
It’s important to see a doctor if you suspect you might have orofacial granulomatosis. They can take a sample of the swollen tissue for testing in a lab to help identify the underlying cause.