Orofacial Granulomatosis

What is orofacial granulomatosis?

Orofacial granulomatosis is a rare skin condition. It may cause lumps that you can see or feel on your face, inner cheeks or lips.

Orofacial granulomatosis that affects only your lips is known as:

• Cheilitis granulomatosa.
• Granulomatous cheilitis.
• Miescher cheilitis.

Who might get orofacial granulomatosis?

Orofacial granulomatosis isn’t common, affecting less than 1% of people. Although it can occur at any age, it’s more common in people who are 20 to 40 years old.

The condition can occur alone (idiopathic) or with other conditions, such as:

• Crohn’s disease.
• Sarcoidosis.
• Infectious diseases, such as tuberculosis.

Orofacial granulomatosis is also often associated with Melkersson-Rosenthal syndrome, a neurological condition that involves three symptoms:

• Swelling on your face or lips.
• Ridges or grooves in your tongue.
• Repeat episodes of facial paralysis (inability to move parts of your face).

What causes orofacial granulomatosis?

Scientists don’t know what causes orofacial granulomatosis. But they’re researching whether it’s related to:

• Allergies or sensitivities to foods such as cinnamon, chocolate and certain preservatives.
• Bacterial or fungal infections.
• Immune responses in people with genetic abnormalities.

They believe that swelling occurs when granulomas block your lymphatic system, leading to lymphedema.

What are the symptoms of orofacial granulomatosis?

The most common early symptom of orofacial granulomatosis is soft, painless swelling of one or both lips. It occurs in your lower lip more often than in your upper lip. The swelling comes and goes, and it can last weeks or months. Swelling may eventually become rubbery, firm, painful and permanent.

Related symptoms may include:

• Cracked lips.
• Drooling.
• Dry, red, peeling skin around your mouth.
• Grooves or ridges on your tongue.
• Mouth ulcers or mucosal tags (bumps or small flaps of extra skin on the tissue inside of your mouth).
• Trouble speaking or eating.
• Swelling or bumpiness of your mouth, cheeks, gums, tongue, chin, eyes, forehead or scalp.

Is orofacial granulomatosis contagious?

Orofacial granulomatosis may spread to other areas of your face, but it isn’t contagious.

How is orofacial granulomatosis diagnosed?

To diagnose this condition, a dermatologist must take a small sample of the swollen tissue (biopsy). A pathologist will analyze the tissue and confirm whether it contains noncaseating granulomas.

The healthcare provider may order other tests to diagnose related conditions, including:

• Blood tests for infectious diseases such as tuberculosis.
• Chest X-rays to look for sarcoidosis.
• Colonoscopy and blood tests to look for Crohn’s disease.
• Patch tests for allergies or sensitivities.

How is orofacial granulomatosis treated?

Your healthcare provider might recommend treatment if the condition causes:

• Concerns about the way you look.
• Pain.
• Trouble using your face or mouth properly.

The most common treatment involves corticosteroids. The type of steroid prescribed depends on how severe the condition is:

• Topical steroids that you put on your skin, such as ointments, creams or mouthwashes.
• Cortisone injections delivered into the swollen area.
• Systemic steroids, usually prednisone, which is a pill that you swallow.

Your healthcare provider also may recommend:

• Antibiotics to fight infections.
• Changes to your diet if any allergies have been identified.
• Oral or topical drugs to suppress the immune system (immunosuppressants).
• Surgery to reduce permanent swelling that interferes with speaking, eating or other important functions.

Is there a cure for orofacial granulomatosis?

Unfortunately, there’s no cure for orofacial granulomatosis yet. Treatments aim to manage symptoms and make people more comfortable.

How can I prevent orofacial granulomatosis?

Because scientists don’t yet understand what causes orofacial granulomatosis, there’s no proven way to prevent it.

What’s the outlook for people with orofacial granulomatosis?

Treatment for orofacial granulomatosis is complex. Current strategies may work slowly, over months or years. Sometimes, they don’t help at all.

Rarely, the condition may suddenly go away on its own (spontaneous remission).

How can I best learn to cope with orofacial granulomatosis?

Living with orofacial granulomatosis can be challenging. You may have trouble coping with the way you look or the way your mouth functions. Some strategies can help you cope with the psychological effects of the condition:

• Counseling sessions to talk about your feelings and ways to handle them.
• Support groups to connect with other people with similar conditions.
• Positive-thinking exercises, such as identifying and concentrating on your best qualities or achievements.
• Social activities with supportive people so you don’t become isolated.

When should I seek medical attention for orofacial granulomatosis?

You should call your healthcare provider right away if you develop weakness or paralysis in any part of your face. Also, report any ridges or grooves in your tongue. These symptoms might be signs of Melkersson-Rosenthal syndrome or another disorder.

A note from Cleveland Clinic

Orofacial granulomatosis is a rare skin condition. It causes swelling in your lips, mouth and other parts of your face. Talk to your healthcare provider if you have any facial swelling so you can be tested and receive treatment. There’s no cure for orofacial granulomatosis, but corticosteroids and other treatments may reduce symptoms.