Parry-Romberg Syndrome (Progressive Hemifacial Atrophy)

What is Parry-Romberg Syndrome?

Parry-Romberg syndrome, also called progressive hemifacial atrophy, is a rare condition in which the tissues of one side of the face gradually waste away. The cause is not known, and the shrinking can involve the fat under the skin, muscle, cartilage and bone.^[1] It needs evaluation and management by physicians, and our role is limited to supportive care.

Common symptoms

The main sign is slowly increasing one-sided facial asymmetry, often beginning as subtle skin tightening, color change or loss of fat in the cheek or around the eye.^[1]

• Hollowing or sinking of one side of the face, sometimes with thinner or darker skin and hair loss
• A sunken eye, differences in eye color or other eye problems^[2]
• Headaches, seizures, or facial nerve pain^{[1, 3]}
• Jaw and dental problems on the affected side^[4]
• Distress, social withdrawal, anxiety and low self-esteem^[1]

Causes and risk factors

The cause is uncertain. Autoimmune, nerve-related and blood-vessel theories have been proposed, but no single mechanism explains every case.^[1] Injury, infection and autonomic nervous system changes have also been suggested.^[3]

Most people first notice changes in the first two decades of life, and the condition is more common in females.^[5] It is often viewed as part of a spectrum with a form of localized scleroderma called linear morphea en coup de sabre, and the two can occur together.^[3]

How it is diagnosed and treated conventionally

Diagnosis rests on an examination of the face and a search for related problems, with blood tests and CT or MRI scans used as needed. A biopsy is rarely needed, but can help separate it from similar conditions.^[1]

The disease usually progresses slowly over 2 to 20 years and then becomes quiet.^[3] During the active phase, physicians may use immune-modulating treatment, with varying success, especially when the condition overlaps with linear scleroderma. Once the condition has stabilized, reconstructive surgery becomes the cornerstone of care, and fat grafting is commonly used for mild to moderate tissue loss.^{[1, 6]} Eye, neurologic, physical therapy and psychological care are added as needed.^[1]

Why Parry-Romberg Syndrome calls for a whole-person approach

Parry-Romberg syndrome affects more than the appearance of the face. Eye, nerve, jaw and emotional health can all be involved, and no standard guidelines for imaging, treatment or follow-up have been defined.^[3] Coordinated care between several specialists is recommended.^[1]

Our supportive care is aimed at some of the surrounding symptoms, not at the shrinking of the tissue.

Neurologic symptoms and headache

Headaches and seizures are well documented, along with migraine and trigeminal neuralgia.^{[1, 3]} Any new neurologic symptom needs a physician's assessment before anything else. Once a neurologist is involved, headache is one of the symptoms for which complementary care has been studied.

Eye and jaw involvement

Eye changes can include sinking of the eye, uveitis and changes in the retina or optic nerve. They need regular ophthalmology review.^[2] Jaw and dental problems on the affected side are part of the picture and are managed by dental and maxillofacial teams.^[4]

Emotional and social effects

The psychosocial effects can be significant. Visible facial change commonly leads to emotional distress and social withdrawal, and psychological counseling contributes to rehabilitation and quality of life.^{[1, 5]} Stress management is therefore a reasonable focus for supportive care.

Acupuncture as supportive care in Parry-Romberg Syndrome

What the research shows

No clinical trials of acupuncture for Parry-Romberg syndrome were found in PubMed, and acupuncture has not been shown to slow or reverse facial atrophy. The evidence below is about headache and pain in other patient groups. It may or may not apply to people with this condition.

For chronic pain, an individual patient data meta-analysis of 39 trials with 20,827 patients found acupuncture better than sham acupuncture or no acupuncture for musculoskeletal pain, osteoarthritis, chronic headache and shoulder pain. The effect over sham was small, and benefit lasted for a year with a modest decrease.^[7]

For tension-type headache, a meta-analysis of 14 trials with 2,795 participants found that acupuncture reduced headache frequency more than sham acupuncture, but the evidence was low to very low quality.^[8] For migraine without aura, a meta-analysis of 14 trials with 1,155 participants found fewer migraine attacks with acupuncture than with sham acupuncture or medication, but the authors judged the evidence mostly low quality and the effectiveness uncertain.^[9]

How acupuncture may work

For chronic pain, the analysis above suggests that benefit is not explained by placebo effects alone, although expectations and treatment context also contribute.^[7] We are not aware of research on how acupuncture might act on the tissues affected in Parry-Romberg syndrome.

What treatment involves

Treatment uses fine, sterile, single-use needles placed at points on the body, usually left in place for about 20 to 40 minutes. A short course of visits is followed by reassessment of headache, sleep and stress. We would be cautious about needling over areas of active skin inflammation or very thin skin on the face, and would discuss this with your treating team.

Serious problems from acupuncture are rare. In prospective studies, about 9 in 100 patients had a minor reaction such as brief bleeding, soreness or redness at a needle site, and serious events occurred in about 1 in 10,000 patients.^[10] Tell the practitioner first if you take a blood thinner, have a bleeding disorder, are pregnant, have a pacemaker (relevant to electroacupuncture), or take immune-suppressing medicines.

Ayurvedic medicine as supportive care in Parry-Romberg Syndrome

The Ayurvedic view

Classical Ayurveda does not describe this condition. A practitioner might describe progressive one-sided tissue loss as depletion of the tissues (dhatu kshaya) with aggravated Vata, the principle said to govern movement and nerve function. This is a traditional framework for individualizing supportive care, not a medical explanation of the disease.

Therapies that may be used

Supportive care is chosen for the individual and may include:

• Dietary guidance such as regular, nourishing, freshly cooked meals and steady sleep hours
• Stress-reducing practices such as breathing exercises and gentle yoga
• Herbs studied for stress, such as ashwagandha, used only after a review of your medicines and with your physician's knowledge
• Gentle warm oil massage on the body, avoiding the affected face unless your specialists agree

Panchakarma procedures are not appropriate during pregnancy, acute illness or frailty, or for anyone with active, untreated disease or on immune-suppressing treatment, unless the treating physician agrees. We would not use them in place of any medical treatment.

What the research shows

No clinical studies of Ayurvedic treatment for Parry-Romberg syndrome were found in PubMed. For stress and anxiety in general, two meta-analyses of ashwagandha (Withania somnifera) trials in adults found lower stress and anxiety scores than with placebo. One pooled 12 trials with 1,002 participants and rated the certainty of the evidence low, with large differences between trials.^[11] The other pooled 9 trials with 558 participants and reported mild to moderate side effects in four of the included studies.^[12]

These trials did not include people with this condition. Both meta-analyses called for further high-quality research.

Herbal medicine as supportive care in Parry-Romberg Syndrome

Herbs and formulas that have been studied

No herbs or formulas have been tested in Parry-Romberg syndrome. Herbal care at our clinic is limited to symptom support, such as ashwagandha for stress as described above, and is prescribed individually.

What the research shows

There is no research on herbal medicine for this condition. The only herbal evidence we cite is the ashwagandha research for stress and anxiety in other populations, which is low in certainty.^{[11, 12]} No herb has been shown to affect the facial atrophy or the immune process thought to be involved.

Safety and herb-drug interactions

Herbs can interact with medicines. Reported interactions include St John's wort lowering blood levels of the immune-suppressing drugs ciclosporin and tacrolimus.^[13] Anyone taking immune-suppressing drugs, anticonvulsants or other prescribed medicines needs a review of every herb and supplement by the prescribing physician and by us before use. The ashwagandha trials above were not done in people with this condition, so how ashwagandha behaves alongside immune-suppressing treatment has not been established.

Herbal and dietary supplements are a recognized cause of liver injury, which matters if methotrexate or similar drugs are being taken.^[14] Ashwagandha in particular has been linked to liver injury in case reports, and the effects were most severe in people who already had chronic liver disease.^[15] About one fifth of Ayurvedic medicines bought online contained detectable lead, mercury or arsenic.^[16] Use only products from tested sources, and tell us if you are pregnant or breastfeeding, or have liver or kidney disease.

Translating traditional medicine into modern biological language

This section is interpretation, not equivalence. Chinese medicine might describe a one-sided loss of facial tissue as poor nourishment of the face from deficient qi and blood or blood stasis in the channels. Ayurveda might describe it as tissue depletion with aggravated Vata. Researchers instead study immune, nerve and blood-vessel mechanisms, none of which fully explains the condition.^[1]

For the symptoms we can support, such as headache and stress, the trials cited above measured symptom relief and did not test any biological explanation. No study has shown that a traditional pattern matches a biological process in Parry-Romberg syndrome.

How this care fits with your medical care

Parry-Romberg syndrome requires specialist medical care. For a condition like this, we ask that you are under a physician's care for it and that your treating team knows you are receiving complementary care. Your team may include dermatology, rheumatology, neurology, ophthalmology, dental and plastic surgery specialists.

Please bring your diagnosis, imaging and eye reports, a list of all medicines and supplements, and details of any planned surgery or fat grafting. We do not diagnose or monitor the disease, and we do not change prescribed treatment. Do not stop immune-suppressing or seizure medicines without your prescriber.

When to seek urgent medical care

New or changing symptoms in this condition need prompt medical assessment, because the disease can involve the brain and eyes.^[1]

• A seizure, especially a first seizure or one that does not stop
• Sudden vision loss, a painful red eye or double vision
• Sudden severe headache, weakness, numbness or trouble speaking
• Rapid worsening of the facial changes, or new patches of tight, hardened skin
• Fever with severe headache or confusion
• Thoughts of harming yourself, which need immediate help from 988, the Suicide and Crisis Lifeline (call or text 988)

For any emergency, call 911 or go to the nearest emergency department.