Parry–Romberg syndrome is a rare condition where one side of the face slowly becomes thinner and smaller over time. The thinning usually starts with the fat just under the skin. The thinning may then involve the skin itself, the muscle underneath, and in some people the facial bones. Doctors call this “progressive hemifacial atrophy,” which simply means “gradual loss of tissue on one half of the face.” The change usually begins in childhood or the teen years, and it tends to progress for several years before it becomes quiet or “burns out.” The condition can be mild and only noticeable in photographs, or it can be more obvious, changing the shape of the cheek, the jaw, the nose, or the area around one eye. Some people also have symptoms beyond the face, such as headaches, facial pain, or seizures, because the same disease process can sometimes involve nerves or the brain.

Parry-Romberg Syndrome (PRS) is a rare condition where one side of the face slowly becomes thinner over time. The skin, the fat under the skin, the muscles, cartilage, and sometimes even the facial bones can shrink. This thinning usually begins in childhood or the teenage years, gets worse slowly for a few years, and then often stops and becomes stable. Many people have symptoms only on one side, but rarely both sides can be affected. Some people can also have eye problems, headaches, or seizures because nearby tissues and nerves may be involved. PRS is sometimes considered part of the “localized scleroderma” family, especially the head-and-face type called “en coup de sabre.” DermNet®EyeWikiBioMed Central

Parry–Romberg syndrome is not the same as simple weight loss, and it is not caused by normal aging. It is also not a cosmetic issue alone, because it can affect function, such as chewing, speaking, or closing the eyelids completely. The exact cause is not known. Many experts think it is related to an immune system problem or a problem with the small blood vessels or nerves that supply the face. There is no single test that proves a person has this condition. Doctors diagnose it by the pattern of changes over time and by ruling out other conditions that can look similar. Treatment focuses on calming the active phase when possible and rebuilding missing volume or structure once the condition is quiet.

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Types

1. By which side and how much of the face is involved. Most people have one side affected (usually the left, but either side can be involved). The area can be small and limited to the cheek, or it can involve the forehead, nose, lips, and jaw. Rarely both sides are involved but often to different degrees.

2. By age at onset. Childhood-onset is most common and often more noticeable because the face grows as the child grows. Adult-onset is less common but can occur and may progress more slowly.

3. By stage of activity. An active phase means the thinning is still progressing and the skin may show color changes or tightness. A burnt-out phase means the changes have stabilized for a year or more with no further loss.

4. By depth of tissue loss. Some people mainly lose subcutaneous fat (giving a hollowed look). Others also lose muscle and bone, which changes the facial outline and bite.

5. With or without skin sclerosis (“morphea”) overlaps. Some patients have streak-like firm or tight skin on the forehead or scalp called linear scleroderma “en coup de sabre.” This is an overlap pattern that belongs to the same family of conditions.

6. By presence of neurologic features. Some have neurologic involvement (headaches, migraine-like symptoms, facial pain, seizures, or white-matter changes on MRI). Others have only facial contour changes.

7. By severity. Mild means subtle asymmetry. Moderate means clear hollowing and contour change. Severe means deep tissue and bone involvement with functional effects like jaw misalignment or eyelid changes.

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Possible causes and contributors

The true cause is still unknown. Most of the ideas below are theories based on patterns doctors have seen. They help guide testing and care, but none of them explains every case.

1. Autoimmune misdirection. The immune system may mistakenly attack tissues of the face, leading to slow loss of fat, skin, and sometimes bone.

2. Inflammation of tiny blood vessels. Small-vessel inflammation (vasculitis-like changes) may reduce blood supply and oxygen to facial tissues, causing gradual atrophy.

3. Nerve-related (trigeminal) changes. The trigeminal nerve supplies sensation and influences blood flow in the face. Disturbance in this system may trigger local tissue loss.

4. Sympathetic nervous system imbalance. Overactivity of the “fight or flight” fibers can tighten small vessels and reduce nutrition to tissues over time.

5. Somatic mosaicism. A small, localized genetic change arising early in development could affect only part of the face, explaining the one-sided pattern.

6. Overlap with localized scleroderma. Some people have features similar to morphea (localized scleroderma), suggesting shared immune pathways that harden or thin tissues.

7. Prior facial trauma. Some patients recall an injury or surgery on the affected side before changes began. Trauma may act as a trigger in susceptible people.

8. Infections as triggers. Past infections, including Borrelia in some reports, have been proposed as triggers, but this is inconsistent and not proven in all patients.

9. Chronic migraine-type vasospasm. Repeated vessel narrowing in migraine biology might reduce blood flow to tissues in some individuals.

10. Inflammatory cytokines. Chemical messengers of inflammation can drive tissue breakdown and remodeling.

11. Autoantibodies. Some patients show positive antinuclear antibodies or other immune markers, hinting at immune system involvement.

12. Endocrine shifts. Puberty-related hormonal changes coincide with onset in many children and adolescents, possibly modulating immune activity.

13. Microvascular clotting tendencies. Rare blood tendencies to form microclots might starve local tissues of nutrients and oxygen.

14. Connective tissue regulation defects. Imbalance in collagen building and breakdown can thin skin and supporting tissues.

15. Dental or jaw triggers. Dental infections or procedures have been temporally associated in some cases, though a direct cause-effect link is not proven.

16. Environmental exposures. Radiation or chemical exposures have been suggested rarely, mainly from case reports.

17. Genetic predisposition. Family history of autoimmune or connective tissue diseases may raise susceptibility, even when the syndrome itself is not inherited.

18. Immune cell infiltration. Biopsies in overlap cases can show immune cells in the skin, indicating an active immune process.

19. Matrix remodeling enzymes. Overactivity of enzymes that break down connective tissue (like MMPs) may thin the skin and fat.

20. Body’s repair response imbalance. The normal repair process may “over-correct” in the wrong direction, leading to net tissue loss rather than restoration.

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Common signs and symptoms

1. Gradual hollowing of one cheek. The cheek on one side looks thinner and flatter as the fat underneath slowly disappears.

2. Skin color and texture changes. The skin can look darker or lighter, feel tighter, or show a shiny surface over the thinning area.

3. Sunken eye on the affected side (enophthalmos). The eye can look more recessed because the fat around the eye has decreased.

4. Asymmetry of the nose or lips. The nose may pull toward the thin side, and the lips can look smaller or uneven.

5. Jaw and bite changes (malocclusion). The upper and lower teeth may no longer meet normally because bone and soft tissue have changed.

6. Visible muscle thinning. Chewing muscles can feel weaker or look smaller on the affected side.

7. Forehead or scalp groove. A line-like depression can appear on the forehead or scalp (especially with en coup de sabre overlap), and hair in that line can thin.

8. Headaches or migraines. Recurrent headaches are common and can be on the same side as the facial changes.

9. Facial pain or tingling. Some people feel burning, aching, or electric shock–like pains along the cheek, jaw, or around the eye.

10. Seizures. A small group of patients develop seizures, often when brain MRI shows changes on the same side as the facial atrophy.

11. Eye surface irritation and dryness. The eyelids may not close fully, or tear film can be unstable, causing dryness and irritation.

12. Tooth root exposure or gum recession. Thinning tissues can make teeth look longer or sensitive.

13. Nasal blockage or deviation. Tissue loss can twist the nasal passages or change airflow on one side.

14. Speech or chewing fatigue. Talking and chewing can feel tiring if muscles are thin or if the bite is misaligned.

15. Psychosocial stress. Changes in appearance can strongly affect confidence, social comfort, and mood, especially in teens.

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Diagnostic approach

There is no single “Parry–Romberg test.” Doctors combine careful examination with targeted tests to confirm the pattern, check disease activity, and rule out other conditions. Below are common tools, grouped by type.

A) Physical examination tests

1. Standard facial inspection and palpation. The clinician looks and gently feels both sides of the face, noting areas of fat loss, tight skin, muscle thinning, and any line-like grooves. This simple exam shows the pattern and depth of change.

2. Tape or caliper facial measurements. The clinician measures distances (for example, cheek width or jaw angles) on both sides. Repeating these measurements over months helps track whether the condition is still progressing.

3. Ophthalmic surface and eyelid closure check. The clinician checks blink function, eyelid closure, and corneal surface with a light. This protects the eye by catching dryness or exposure problems early.

4. Dental and occlusion screening. The clinician looks inside the mouth for gum recession, tooth sensitivity, and how upper and lower teeth meet. This helps detect functional effects that may need early dental or orthodontic care.

B) Manual (bedside) functional tests

5. Masseter and temporalis muscle strength test. The clinician asks you to clench and chews against resistance, feeling the muscle bulk on both sides. This shows loss of chewing power on the thin side.

6. Temporomandibular joint (TMJ) range-of-motion test. Opening, closing, and moving the jaw side to side are checked by hand. Limited or uneven motion suggests joint strain from asymmetry.

7. Cranial nerve sensory mapping. Light touch and pinprick are tested in the three trigeminal nerve zones (forehead, cheek, jaw) to look for areas of reduced or altered sensation.

8. Smile and facial movement symmetry test. The clinician observes how the mouth corners and eyelids move during smiling, frowning, and blinking. This reveals subtle functional differences and helps guide therapy.

C) Laboratory and pathological tests

9. Antinuclear antibody (ANA) and autoimmune screen. Blood tests look for immune markers sometimes seen in connective tissue conditions. A positive result supports, but does not prove, an autoimmune contribution.

10. Inflammatory markers (ESR, CRP). These tests show whether systemic inflammation is present. They can guide whether immune-calming treatment should be considered during active phases.

11. Thyroid function tests. Thyroid imbalance can worsen hair and skin changes and should be ruled out as a contributor.

12. Infectious triggers as indicated (for example, Lyme serology where epidemiologically relevant). Testing is tailored to your location and risk factors to look for treatable triggers, recognizing that most patients will not have an infectious cause.

13. Skin or tissue biopsy when diagnosis is unclear or morphea overlap is suspected. A small sample of affected skin can show thickened collagen, inflammation, or other features that support the diagnosis and guide therapy.

D) Electrodiagnostic and neurophysiologic tests

14. Electroencephalogram (EEG) for those with seizures or unusual spells. EEG looks for abnormal brain electrical activity, helps classify seizures, and guides anti-seizure treatment if needed.

15. Blink reflex or trigeminal nerve studies in selected cases. Specialized tests check the pathways that control blinking and facial sensation to detect subtle nerve involvement.

16. Visual evoked potentials when visual symptoms are present. This test measures how quickly the brain responds to visual signals and can reveal pathway irritation on the affected side.

E) Imaging tests

17. MRI of the brain and face with contrast. MRI can show white-matter changes, inflammation, or scarring on the same side as the facial thinning. It also assesses the orbit (eye socket) and soft tissues.

18. Maxillofacial CT or cone-beam CT (CBCT). These scans show fine bone detail of the jaw, cheekbones, and nasal structures. They help dentists and surgeons plan bite correction or reconstructive procedures.

19. High-frequency skin and soft-tissue ultrasound. Ultrasound can measure the thickness of skin and fat layers in a painless way and can be repeated to monitor activity over time.

20. Dental panoramic X-ray (orthopantomogram) and orthodontic imaging. These images show tooth roots, jaw growth, and areas of bone change. They help decide on braces, splints, or timing of surgery.

Non-pharmacological treatments (therapies and others)

Notes: These support comfort, function, or appearance. They do not replace medical treatment when the disease is active. Many are best done by a multidisciplinary team (dermatology, plastic/craniofacial surgery, ophthalmology, neurology, dentistry/orthodontics, psychology).

1. Regular monitoring with photos and measurements – Purpose: track pace of change and decide when to treat and when surgery is safe. Mechanism: objective documentation of symmetry, skin firmness, and eye position guides timing. DermNet®

2. Eye surface care (artificial tears, eyelid hygiene, nighttime eye shield if needed) – Purpose: protect the eye if it’s more exposed or dry due to sunken tissues. Mechanism: lubrication reduces friction and dryness injury. EyeWiki

3. Headache and seizure self-care plans – Purpose: reduce triggers (sleep loss, dehydration, screen glare). Mechanism: lifestyle routines ease brain irritability; medical therapy is added when needed. PubMed Central

4. Skin care and sun protection – Purpose: gentle cleansers, moisturizers, and sunscreen to protect sensitive skin. Mechanism: lowers irritation and post-inflammatory color change. DermNet®

5. UVA1/phototherapy (specialist-supervised) – Purpose: calm localized scleroderma-type inflammation in the active phase, sometimes used when deeper disease is present. Mechanism: UVA1 can modulate immune cells and reduce collagen over-production. Evidence exists in morphea; results vary, and it’s not for everyone. PubMedPubMed CentralFrontiers

6. Camouflage makeup/dermal fillers (temporary) – Purpose: short-term improvement of shallow depressions while disease is monitored. Mechanism: optical blending with pigments; hyaluronic-acid fillers add volume for months. (Used cautiously in active disease.)

7. Speech and swallowing therapy (if mouth opening is tight) – Purpose: improve oral function, articulation, and safe eating. Mechanism: targeted stretches and techniques for jaw and soft tissues.

8. Jaw and temporomandibular joint (TMJ) therapy – Purpose: reduce biting discomfort, improve symmetry. Mechanism: bite splints, gentle exercises, and, later, orthodontics.

9. Dental/orthodontic care – Purpose: manage tooth alignment, root resorption risks, and bite differences on the affected side. Mechanism: staged orthodontics after disease stabilization; careful timing is key. Annals of Translational Medicine

10. Psychological support and peer groups – Purpose: address body-image stress, anxiety, or social challenges. Mechanism: counseling, CBT, and connection to rare-disease communities reduce distress. National Organization for Rare Disorders

11. Protective eyewear and helmets (sports) – Purpose: reduce facial/eye trauma risk on the fragile side. Mechanism: simple mechanical protection.

12. Nutrition counseling – Purpose: support healthy weight and wound healing before/after procedures. Mechanism: adequate protein, vitamins, minerals aid tissue repair.

13. 3-D imaging and planning – Purpose: plan future reconstruction and track changes over time. Mechanism: precise surface scans guide safe surgery timing and volume needs. PubMed Central

14. Scar and skin-texture care – Purpose: gentle silicone gel/sheets and massage for cosmetic comfort. Mechanism: improves feel/appearance of superficial linear bands.

15. Dry-eye prevention if eyelids don’t close fully during sleep – Purpose: avoid corneal injury. Mechanism: thicker ointments, eye shields.

16. School/work accommodations – Purpose: reduce stigma and fatigue. Mechanism: flexibility for appointments; permission for hats/eyewear for photosensitivity.

17. Sleep hygiene – Purpose: better headache control and mood. Mechanism: steady sleep schedule reduces neurologic triggers. PubMed Central

18. Physiotherapy for neck/shoulder balance – Purpose: manage posture changes from asymmetry. Mechanism: targeted strengthening and stretching.

19. Sunken eye (enophthalmos) temporary optical aids – Purpose: reduce double-vision or glare while awaiting reconstruction. Mechanism: tailored lenses/tints from ophthalmology. EyeWiki

20. Timing strategy: delay major reconstruction until stable – Purpose: improve long-term results and reduce repeat surgeries. Mechanism: most teams aim for 1–2 years of clinical stability before permanent reconstruction, while temporary measures bridge appearance needs. PubMed Central

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Drug treatments

Important: Doses below are typical adult ranges. Pediatric dosing is weight/size-based and must be individualized by a specialist. Many of these are off-label for PRS but supported by studies and case series in localized scleroderma, en coup de sabre, or PRS overlap.

1. Methotrexate (MTX) – antimetabolite DMARD
   Dose/time: 15–25 mg once weekly (oral or subcutaneous) with folic acid; often combined with a steroid for the first 2–3 months, then continued 6–12+ months.
   Purpose: first-line to quiet active inflammation and stop progression.
   Mechanism: reduces immune cell activity and fibrotic signaling.
   Side effects: nausea, mouth sores, liver enzyme rise; avoid pregnancy and alcohol excess; lab monitoring required. PubMed Central+1

2. Prednisone or pulsed methylprednisolone – corticosteroids
   Dose/time: prednisone ~0.5–1 mg/kg/day tapered; or IV methylprednisolone pulses (e.g., 1 g daily for 3 days) early in active disease.
   Purpose: rapid inflammation control while slower MTX starts working.
   Mechanism: broad anti-inflammatory effect.
   Side effects: mood change, weight gain, blood sugar rise, bone thinning with long use—use the lowest effective dose. PubMed Central+1

3. Mycophenolate mofetil (MMF) – immunosuppressant
   Dose/time: 1–1.5 g twice daily (adults); months of therapy.
   Purpose: alternative to or after MTX, especially in refractory cases.
   Mechanism: inhibits lymphocyte proliferation.
   Side effects: GI upset, infection risk; pregnancy risks; labs needed. Evidence supports benefit in severe or MTX-resistant localized scleroderma. PubMed Central+1

4. Tocilizumab – IL-6 inhibitor biologic
   Dose/time: 162 mg subcutaneous weekly (or IV 8 mg/kg monthly) in reports.
   Purpose: option for refractory head/face linear scleroderma or PRS overlap with neuro/skin activity.
   Mechanism: blocks IL-6 signaling that drives inflammation and fibrosis.
   Side effects: infection risk, liver enzyme rise, lipid changes; monitor labs. Case reports/series show improvement in difficult cases. jaadcasereports.org+1reumatologiaclinica.org

5. Rituximab – B-cell depleting biologic
   Dose/time: 1,000 mg IV on days 1 and 15 (or 375 mg/m² weekly ×4), repeated per specialist.
   Purpose: considered in severe refractory cases with autoimmune features.
   Mechanism: removes B-cells that can drive autoimmunity.
   Side effects: infusion reactions, infection risk; pre-screen for hepatitis. Evidence mainly from case series in morphea/systemic sclerosis and select PRS overlaps. JAMA NetworkPubMed Central

6. Hydroxychloroquine – antimalarial immunomodulator
   Dose/time: 200–400 mg daily; months to years.
   Purpose: mild/moderate inflammatory activity or as a steroid-sparing add-on.
   Mechanism: dampens antigen processing and cytokine release.
   Side effects: rare retinal toxicity—baseline and periodic eye exams needed. Used in localized scleroderma variants though evidence is modest. ScienceDirect

7. Azathioprine – immunosuppressant
   Dose/time: 1–2.5 mg/kg/day; labs needed; TPMT testing may guide risk.
   Purpose: alternative when MTX/MMF cannot be used.
   Mechanism: inhibits purine synthesis in lymphocytes.
   Side effects: low blood counts, infection risk, liver enzyme rise.

8. Cyclosporine – calcineurin inhibitor (systemic)
   Dose/time: ~2–3 mg/kg/day; short-term use with careful monitoring.
   Purpose: rescue therapy in highly active, refractory disease.
   Mechanism: reduces T-cell activation.
   Side effects: kidney stress, blood-pressure rise, gum changes.

9. Intravenous immunoglobulin (IVIG)
   Dose/time: common dermatology practice is 2 g/kg per cycle over 2–5 days monthly; duration individualized.
   Purpose: immunomodulation in refractory cases (especially if steroids/DMARDs fail or are not tolerated).
   Mechanism: complex—neutralizes autoantibodies, modulates Fc receptors and cytokines.
   Side effects: headache, thrombotic risk (rare); requires infusion center. ScienceDirectPubMed Central

10. Topical tacrolimus or high-potency topical steroids (adjuncts)
    Dose/time: tacrolimus 0.1% ointment twice daily to superficial active bands; course varies.
    Purpose: local symptom relief in superficial linear lesions; limited effect in deep PRS.
    Mechanism: reduces local T-cell activity.
    Side effects: local burning; use under dermatology guidance. PubMed Central

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Dietary molecular supplements

These do not treat or cure PRS. They may support overall health, skin healing, or inflammation balance. Always review supplements with your clinician, especially if you take MTX, MMF, biologics, or blood thinners.

1. Vitamin D3 – 1,000–2,000 IU/day (adjust to blood level). Supports immune balance and bone health; low levels are common in chronic inflammatory conditions.

2. Omega-3s (EPA/DHA) – 1–2 g/day combined EPA+DHA. Anti-inflammatory lipid mediators may ease general inflammation and support cardiovascular health.

3. Curcumin (turmeric extract, standardized) – 500–1,000 mg/day with piperine or a bioavailable form. May down-regulate NF-κB and fibrotic signaling; watch for anticoagulant interactions.

4. N-acetylcysteine (NAC) – 600–1,200 mg/day. Antioxidant and glutathione precursor; may help oxidative stress.

5. Alpha-lipoic acid – 300–600 mg/day. Antioxidant that may support nerve comfort in neuropathic pain syndromes.

6. Coenzyme Q10 – 100–200 mg/day. Mitochondrial support; may aid statin users or fatigue.

7. Collagen peptides – 5–10 g/day. Provides amino acids (glycine, proline) for connective-tissue support (adjunct for skin/hair/nail health).

8. Hyaluronic acid (oral) – 120–240 mg/day. Hydration support for skin; effects are mild.

9. Resveratrol – 150–300 mg/day. Antioxidant/anti-fibrotic signals in preclinical data; human evidence limited.

10. Probiotics (multi-strain, 10–20 billion CFU/day) – Gut support; may help general immune tone. Choose reputable brands; stop if bloating persists.

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Regenerative / stem-cell related” options

Important safety note: There are no approved stem-cell drugs for PRS. The items below are advanced immunotherapies or investigational regenerative approaches used only by specialists, usually for refractory disease, and often within clinical trials. They can carry meaningful risks and costs.

1. Intravenous Immunoglobulin (IVIG) – 2 g/kg per cycle monthly. Mechanism: broad immune modulation (anti-cytokine, anti-idiotype). Role: refractory localized scleroderma/PRS overlap. Risks: infusion reactions, rare thrombosis. Evidence base: case series and reviews in scleroderma contexts. ScienceDirectPubMed Central

2. Rituximab (B-cell depletion) – dosing as above. Mechanism: removes B cells that can drive autoimmunity. Role: selected refractory cases with autoimmune features; evidence mostly extrapolated from systemic sclerosis and case reports. Risks: infections, infusion reactions. JAMA NetworkPubMed Central

3. Tocilizumab (IL-6 blockade) – 162 mg SC weekly/8 mg/kg IV monthly in reports. Mechanism: dampens IL-6-driven inflammation/fibrosis. Role: refractory en coup de sabre/PRS with skin and sometimes neurologic activity; case reports/series show benefit in some patients. Risks: infection, lab changes. jaadcasereports.orgreumatologiaclinica.org

4. Abatacept (CTLA-4-Ig; T-cell co-stimulation blocker) – 125 mg SC weekly or IV weight-based. Mechanism: reduces T-cell activation. Role: small case reports/series suggest benefit in severe morphea variants; used when MTX/MMF fail. Risks: infection, headache. PubMed CentralNHS England

5. Adipose-derived cell-enriched fat grafting (investigational biologic augmentation of fat transfer) – Not a drug; combines standard fat graft with concentrated adipose-derived stromal cells. Mechanism: adds volume and may improve tissue quality via paracrine repair signals. Role: experimental; confined to specialized centers and studies; long-term safety/effectiveness still being defined. Oxford Academic

6. Mesenchymal stromal cell (MSC) therapies (experimental) – Doses and routes vary by trial; not standard care. Mechanism: immune modulation and anti-fibrotic paracrine effects. Role: only in clinical trials for scleroderma-spectrum diseases; talk to tertiary centers if interested. Risks: unknown long-term effects; regulatory oversight essential. (General scleroderma trials exist; PRS-specific data are lacking.)

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Surgeries/procedures

Most surgeons prefer 1–2 years of no change before permanent reconstruction, but individual needs vary (for severe psychosocial impact, earlier staged procedures may be discussed). Multiple touch-ups are common over time. PubMed Central

1. Autologous fat grafting (lipofilling; “Coleman technique”) – Fat is gently harvested from your abdomen or thigh, processed, and injected under the skin to restore contour. Why done: it is the least invasive volume-restoring option and can be repeated. Over time, some fat resorbs, so top-ups may be needed. Lippincott JournalsPubMed Central

2. Microvascular free-flap transfer (e.g., parascapular/scapular/serratus flaps) – A tissue flap with its artery/vein is moved from the back or side to the face and connected under a microscope. Why done: provides larger, more durable volume for severe atrophy and can improve soft-tissue quality. PubMed CentralPubMedScienceDirect

3. Alloplastic facial implants (custom or standard silicone/porous polyethylene) – Solid implants are placed to rebuild cheek, chin, or orbital rims. Why done: adds structure when soft-tissue alone is not enough. Surgeons weigh risks like infection or movement of the implant. Lippincott Journals

4. Orthognathic (jaw) surgery and dental/orthodontic reconstruction – After growth and disease stabilization, planned jaw repositioning and staged orthodontics can improve bite and symmetry. Why done: corrects functional chewing problems and facial balance. Annals of Translational Medicine

5. Orbital reconstruction for sunken eye (enophthalmos) – Options include orbital volume implants or combined soft-tissue augmentation. Why done: protect the eye surface, improve symmetry, and reduce double-vision risk. EyeWiki

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Prevention tips

We cannot fully prevent PRS, but we can lower risks of complications and support quality of life.

1. Seek early evaluation if new facial changes appear; early immune-calming therapy may reduce damage. PubMed Central

2. Keep regular follow-ups (dermatology, plastics, neuro, eye, dental).

3. Protect the eye on the affected side from dryness and trauma. EyeWiki

4. Maintain good oral hygiene and routine dental/orthodontic checks. Annals of Translational Medicine

5. Use sunscreen and gentle skin care to limit irritation. DermNet®

6. Sleep well, hydrate, and manage stress to help headaches and general wellbeing. PubMed Central

7. Vaccinate appropriately, especially if you’re on immunosuppressive medicines (ask your doctor which vaccines are safe/live vs non-live).

8. Avoid smoking and limit alcohol to support skin healing and medication safety.

9. Use protective gear for high-impact sports.

10. Keep a symptom diary (photos, eye comfort, headaches) to spot flares early.

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When to see a doctor urgently

• New or fast-worsening facial asymmetry; new tender, firm skin bands or color change on the face/scalp.

• New headaches, seizures, or vision changes (blur, pain, double-vision, red eye). EyeWikiPubMed Central

• Eye that looks more sunken or does not close fully; new light sensitivity or dryness. EyeWiki

• Jaw pain, trouble chewing, or sudden bite changes. Annals of Translational Medicine

• Side effects from medicines (fever, cough, shortness of breath, severe nausea, yellowing of the eyes/skin, severe headache).

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What to eat” and “what to avoid

What to eat (5):

1. Protein-rich foods (fish, eggs, beans, yogurt) to support tissue repair.

2. Omega-3 sources (fatty fish, flax, walnuts) for inflammation balance.

3. Colorful fruits/vegetables for antioxidants and vitamins A/C/E.

4. Whole grains and legumes for steady energy and fiber.

5. Calcium- and vitamin-D-rich foods (dairy or fortified alternatives) for bone health, especially if you’ve used steroids.

What to limit/avoid (5):

1. Ultra-processed foods high in sugar and trans fats (worsen inflammation).

2. Excess salt (swelling, blood-pressure issues with some drugs).

3. Excess alcohol (liver risk with MTX/MMF; ask your clinician).

4. Grapefruit with certain medicines (possible interactions—check your list).

5. High-dose herbal “immune boosters” without supervision (can clash with immunosuppressants).

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Frequently Asked Questions

1) Is PRS life-threatening?
Usually no. It mainly changes facial shape and sometimes affects eye or nerves. Serious problems (like seizures) need careful care but are manageable. EyeWiki

2) Does PRS always keep getting worse?
No. It often worsens slowly for a few years, then reaches a stable phase with little change. That’s when surgeons prefer to do permanent reconstruction. EyeWiki

3) Is PRS the same as scleroderma?
Not exactly. Many experts see PRS as overlapping with localized scleroderma (morphea), especially “en coup de sabre.” Treatments often follow localized scleroderma guidance. BioMed Central

4) How do doctors know if it is “active”?
New changes (color, firmness, tenderness), recent photos showing progression, or MRI signs can point to activity. In that case, immune-calming medicines are considered. PubMed Central

5) What is first-line medicine?
Methotrexate plus a short course of steroids is common first-line for active disease, based on localized scleroderma evidence. PubMed Central

6) If methotrexate doesn’t work, what’s next?
Options include mycophenolate, IVIG, or biologics like tocilizumab or rituximab in tough cases—always specialist-directed. PubMed Centraljaadcasereports.org

7) Can light therapy help?
UVA1 can help some localized scleroderma patients; benefit varies and deeper PRS may respond less. It must be supervised. PubMedPubMed Central

8) When is surgery appropriate?
Often after at least 1–2 years of stability; earlier staged work can be considered for severe psychosocial impact. Multiple touch-ups are normal. PubMed Central

9) What surgeries are most used?
Autologous fat grafting for mild-to-moderate volume loss; free-flap tissue transfer for severe cases; implants for structure; jaw and orbital reconstructions as needed. Lippincott JournalsPubMed Central

10) Will the fat graft “last”?
Some of the transferred fat survives long-term; some resorbs. Many people need 1–3 sessions over time for best balance. Lippincott Journals

11) Are stem-cell injections standard care?
No. They are experimental. If you’re interested, talk to a major academic center about clinical trials and risks. (Do not purchase unregulated “stem-cell” treatments.)

12) Can PRS affect the brain?
Some patients have MRI changes, headaches, or seizures. A neurologist guides imaging and treatment if symptoms appear. PubMed Central

13) Will my eye be okay if it looks sunken?
Ophthalmologists can protect the surface with drops/ointments and later coordinate orbital reconstruction to restore support. EyeWiki

14) Can children get orthodontics with PRS?
Yes—timing and planning are very important and usually happen after stability, often with staged approaches. Annals of Translational Medicine

15) Where can families find support?
National rare-disease groups and hospital-based craniofacial teams can connect you with resources, counseling, and reconstructive options. National Organization for Rare Disorders

Disclaimer: Each person’s journey is unique, treatment plan, life style, food habit, hormonal condition, immune system, chronic disease condition, geological location, weather and previous medical  history is also unique. So always seek the best advice from a qualified medical professional or health care provider before trying any treatments to ensure to find out the best plan for you. This guide is for general information and educational purposes only. Regular check-ups and awareness can help to manage and prevent complications associated with these diseases conditions. If you or someone are suffering from this disease condition bookmark this website or share with someone who might find it useful! Boost your knowledge and stay ahead in your health journey. We always try to ensure that the content is regularly updated to reflect the latest medical research and treatment options. Thank you for giving your valuable time to read the article.

The article is written by Team RxHarun and reviewed by the Rx Editorial Board Members

Last Updated: August 20, 2025.