Parry-Romberg Syndrome

Parry-Romberg syndrome (PRS) is a rare condition that affects your facial skin, fat, muscles and sometimes bone. It causes slow, gradual shrinkage (atrophy) of your skin and soft tissue on one side of your face. It usually starts in childhood or the teen years. It’s more common in females than males.

Symptoms often begin with changes in your skin color. Over time, your face may lose fat and look thinner on one side. You might also develop seizures, migraines or jaw problems.

You may hear your provider call PRS:

• Progressive hemifacial atrophy
• Progressive facial hemiatrophy
• Idiopathic hemifacial atrophy

They all refer to the same condition.

Treatment manages symptoms. Your provider can help you understand your options and what to expect.

Parry-Romberg syndrome symptoms

PRS can affect your face, eyes and mouth. Symptoms vary from person to person and may include:

• Burning or prickling sensations (facial paresthesia)
• Facial pain
• Headaches and migraines
• Seizures
• Sharp nerve pain in your face (trigeminal neuralgia)
• Hair loss in the affected areas

Eye symptoms may include:

• Blurred or decreased vision
• Double vision
• Eye discomfort from dryness or exposure

Symptoms that affect your mouth may include:

• Bite problems that make chewing harder
• Difficulty speaking clearly
• Jaw pain
• Trouble opening or closing your jaw (trismus)

Parry-Romberg syndrome causes

Healthcare providers don’t know the exact cause, but there are several theories about why people may get this disease. Possible causes include:

• Autoimmune disorders
• Trauma to the face (either accidental or from surgery)
• Genetics
• Certain viral or bacterial infections
• Problems with your brain and nerves (nervous system disorders)

Of these causes, the most widely supported theory is that PRS may be an autoimmune disorder. An autoimmune disorder happens when your immune system mistakenly attacks healthy tissue. PRS often overlaps with a condition called linear scleroderma. Linear scleroderma is an autoimmune disease that affects the skin.

Providers sometimes diagnose this condition in people who also have autoimmune conditions like:

• Scleroderma
• Ankylosing spondylitis
• Inflammatory bowel disease
• Lupus
• Rheumatoid arthritis
• Thyroid disorders, like Hashimoto disease
• Vitiligo

Researchers continue to study PRS to better understand why it develops.

Complications

PRS may lead to complications over time. These could include:

• Bite problems where your upper and lower teeth don’t line up correctly
• Trouble speaking
• Swelling and inflammation inside your eye (uveitis)
• A sunken eye appearance (enophthalmos)
• Dry eye
• Vision changes
• Seizures
• Migraines
• Changes in the way your face looks
• Mental and emotional health challenges

Severe complications are rare, but they may include:

• Blood vessel problems in your brain (aneurysms)
• Decrease in the size of part of your brain (brain atrophy)
• Glaucoma
• Inflammation of blood vessels in your eye (retinal vasculitis)
• Retina pulls away from the back of your eye (retinal detachment)
• Severe vision loss

How doctors diagnose Parry-Romberg syndrome

A healthcare provider will diagnose PRS mainly by examining your face and reviewing your medical history.

During the exam, your provider will look for slow, progressive thinning on one side of your face. They’ll also ask about other symptoms, like headaches, facial pain or problems with vision.

They may order imaging tests, like an MRI or CT scan of your head, to check your brain, bones and blood vessels. These tests help your provider see how much tissue the condition affects and look for complications. An eye doctor may also check your vision and eye health.

There isn’t a specific lab test for PRS, but your provider may order blood tests to look for autoimmune conditions. In rare cases, they may perform a skin biopsy to rule out similar conditions.

Providers may also describe PRS as mild, moderate or severe. These describe how much the condition affects your tissue and bone.

Treatment for Parry-Romberg syndrome

There isn’t a cure for PRS, but treatment may help slow the disease and manage symptoms in the early, progressive phase. After this phase, when the disease is no longer active, the goal of treatment is to improve the appearance and function of the affected tissues.

Your provider will tailor treatment to your symptoms, the stage and your personal goals.

Medications

If PRS is progressing, your care team will focus on calming your immune system with one of the following medications:

• Methotrexate, often used as a first-line treatment
• Corticosteroids, like prednisone, to reduce inflammation
• Other immune-suppressing medications if symptoms are severe or don’t improve

There are also symptom-related treatments available, like:

• Anti-seizure medications if seizures occur
• Migraine treatments for headaches
• Steroid eye drops or other eye treatments for inflammation or glaucoma
• Dental or orthodontic care for bite problems
• Mental health support if anxiety or depression develop

Reconstructive options

Once the condition is stable (for at least one to two years), you might choose to have cosmetic treatments to improve your facial symmetry (evenness) and function. These treatments may even include surgery. Options may include:

• Fat grafting
• Fillers
• Laser treatment
• Implants
• Bone or jaw surgery in more severe cases

When should I see my healthcare provider?

You should see a healthcare provider if you notice:

• Eye symptoms (like blurred vision, pain or double vision)
• Neurologic symptoms (like seizures, severe headaches or strong facial pain)
• New or worsening facial changes (like thinning on one side of your face or a sunken eye)
• Rapid changes (like your face changing quickly over weeks or months)
• Trouble with daily function (like difficulty chewing, speaking or moving your jaw)

Early care may help your provider monitor the condition and start treatment if needed.

What can I expect if I have this condition?

PRS usually progresses slowly over several years. Changes may continue for two to 20 years. Then, it often reaches a stable or inactive phase. It typically doesn’t continue getting worse forever, although rare cases can reactivate later in life.

PRS often begins when your body and identity are still developing. Your appearance can feel especially important. Going through visible facial changes during these years may feel confusing, frustrating or emotionally overwhelming. Many people find counseling or peer support helpful as they adjust.

Over time, you can expect some degree of permanent facial asymmetry (unevenness). The severity varies from person to person. People who develop PRS at a younger age or who have deeper tissue involvement may notice more visible changes. Reconstructive surgery might improve appearance, but it will often require more than one procedure, and results can vary.

Parry-Romberg syndrome life expectancy

This condition doesn’t usually affect life expectancy. It’s chronic, which means it can last for many years, but it’s not typically fatal. Most people with PRS live a normal lifespan.