Primary non-essential cutis verticis gyrata

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ORPHA:357225OMIM:219300Q82.8
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8Treatment centers

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What is Primary non-essential cutis verticis gyrata?

Primary non-essential cutis verticis gyrata (CVG) is a rare skin condition that affects the scalp. In this condition, the skin on the top of the head develops thick folds and deep furrows that look similar to the ridges and grooves on the surface of the brain. The term 'non-essential' means that this form of CVG occurs on its own, without being linked to other underlying medical conditions or syndromes. It is considered 'primary' because it is not caused by another disease process such as a tumor, inflammation, or metabolic disorder. The folds typically run from the front to the back of the scalp and cannot be flattened by pressing on them. The condition is mostly a cosmetic concern, though the deep folds can sometimes trap moisture and lead to skin infections, unpleasant odor, or irritation if not properly cared for. Some people may also experience hair thinning in the affected areas. Primary non-essential CVG is much more common in males than females and usually becomes noticeable around or after puberty. The exact cause is not fully understood, but it may involve overgrowth of the scalp skin. Treatment is generally not required unless the person is bothered by the appearance or develops complications like recurrent infections. When treatment is pursued, surgical removal of the excess skin folds is the main option. There are no medications that can reverse the condition.

Key symptoms:

Thick folds or ridges on the scalpDeep grooves or furrows on the top of the headScalp skin that cannot be flattened when pressedScalp folds that run from front to backHair thinning or hair loss in the folded areasTrapped moisture between scalp foldsUnpleasant odor from the scalp foldsSkin irritation or redness between foldsRecurrent scalp infectionsSelf-consciousness about scalp appearance

Clinical phenotype terms (16)— hover any for plain English
  • Cutis gyrata of scalpHP:0010541
  • Abnormal forehead morphologyHP:0000290
  • GliosisHP:0002171
  • Borderline intellectual disabilityHP:0006889
  • Periventricular leukomalaciaHP:0006970
  • Abnormality of pattern visual evoked potentialsHP:0030455
  • KeloidsHP:0010562
Inheritance
Variable
Can be inherited in different ways depending on the underlying gene
Age of Onset
Juvenile
Begins in the teen years
Orphanet ↗OMIM ↗NORD ↗

Treatments

Source: openFDA + DailyMed · NDA / BLA labels with structured indications · refreshed weekly

No FDA-approved treatments are currently listed for Primary non-essential cutis verticis gyrata.

View clinical trials →

Source: ClinicalTrials.gov · synced daily · phases, status, and PI names normalized at ingest

No actively recruiting trials found for Primary non-essential cutis verticis gyrata at this time.

New trials open frequently. Follow this disease to get notified.

Search ClinicalTrials.gov ↗Join the Primary non-essential cutis verticis gyrata community →

Source: NPI Registry + PubMed · trial PI roles cross-referenced with ClinicalTrials.gov · ranked by match score (publications + PI activity + community signal)

No specialists are currently listed for Primary non-essential cutis verticis gyrata.

View NORD Rare Disease Centers ↗Undiagnosed Disease Network ↗

Treatment Centers

8 centers

Source: NORD Rare Disease Centers + NIH Undiagnosed Diseases Network (UDN) · centers verified active within last 12 months

🏨 Children's

Children's Hospital Colorado Rare Disease Program

Children's Hospital Colorado

📍 Aurora, CO

👤 Boston Children's Hospital Rare Disease Program

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Boston Children's Hospital Rare Disease Program

Boston Children's Hospital

📍 Boston, MA

👤 Boston Children's Hospital Rare Disease Program

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

🏨 Children's

Ann & Robert H. Lurie Children's Hospital Genetics

Lurie Children's Hospital

📍 Chicago, IL

👤 Boston Children's Hospital Rare Disease Program

🏥 NORD

Cincinnati Children's Hospital Medical Center

Cincinnati Children's

📍 Cincinnati, OH

👤 Boston Children's Hospital Rare Disease Program

🏨 Children's

Nationwide Children's Hospital Rare Disease Center

Nationwide Children's Hospital

📍 Columbus, OH

👤 Boston Children's Hospital Rare Disease Program

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

Travel Grants

No travel grants are currently matched to Primary non-essential cutis verticis gyrata.

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Latest news about Primary non-essential cutis verticis gyrata

Source: PubMed + NIH RePORTER + openFDA + clinical-journal RSS · last 30 days · disease-tagged at ingest by AI extraction with human QC

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Questions for your doctor

Bring these to your next appointment

  • Q1.Have you ruled out all other possible causes for the scalp folds?,Do I need any blood tests or imaging to make sure there is no underlying condition?,What is the best way to care for my scalp to prevent infections?,Am I a candidate for surgical correction, and what are the risks?,Could the folds come back after surgery?,Should I see a counselor or psychologist for the emotional impact of this condition?,Is there any chance this condition is related to a genetic syndrome that could affect other parts of my body?

Common questions about Primary non-essential cutis verticis gyrata

What is Primary non-essential cutis verticis gyrata?

Primary non-essential cutis verticis gyrata (CVG) is a rare skin condition that affects the scalp. In this condition, the skin on the top of the head develops thick folds and deep furrows that look similar to the ridges and grooves on the surface of the brain. The term 'non-essential' means that this form of CVG occurs on its own, without being linked to other underlying medical conditions or syndromes. It is considered 'primary' because it is not caused by another disease process such as a tumor, inflammation, or metabolic disorder. The folds typically run from the front to the back of the s

At what age does Primary non-essential cutis verticis gyrata typically begin?

Typical onset of Primary non-essential cutis verticis gyrata is juvenile. Age of onset can vary across affected individuals.

Frequently asked questions about Primary non-essential cutis verticis gyrata

Auto-generated from canonical disease facts (Orphanet, OMIM, ClinicalTrials.gov, openFDA, NPPES). Not a substitute for clinical guidance.

  1. What is Primary non-essential cutis verticis gyrata?

    Primary non-essential cutis verticis gyrata is a rare disease catalogued in international rare-disease ontologies (Orphanet ORPHA:357225, OMIM 219300). It is typically inherited as variable. Age of onset is generally juvenile. For verified primary sources, see the UniteRare Primary non-essential cutis verticis gyrata page.

  2. How is Primary non-essential cutis verticis gyrata inherited?

    Primary non-essential cutis verticis gyrata follows variable inheritance. Genetic counseling is recommended for affected families to understand recurrence risk in offspring and the likelihood of unaffected siblings being carriers. Variants in the underlying gene(s) may be identified via clinical genetic testing.

  3. Are there FDA-approved treatments for Primary non-essential cutis verticis gyrata?

    Approved treatments for Primary non-essential cutis verticis gyrata are tracked from openFDA and DailyMed primary sources. Many rare diseases have no specific FDA-approved therapy; for those, supportive care and management of complications form the basis of clinical care. Orphan-drug-designation status is noted where applicable.

  4. Are there clinical trials for Primary non-essential cutis verticis gyrata?

    Active clinical trials for Primary non-essential cutis verticis gyrata are tracked daily from ClinicalTrials.gov. Trial availability changes frequently; check the UniteRare trial listings for the current count and recruitment status. Sponsors of rare-disease research often welcome inquiries even when a trial is not actively recruiting at a given moment.

  5. How do I find a specialist for Primary non-essential cutis verticis gyrata?

    Verified Primary non-essential cutis verticis gyrata specialists are identified through ClinicalTrials.gov principal-investigator records, peer-reviewed publication authorship (via PubMed), and the NPPES NPI registry. NORD-designated Centers of Excellence and NIH-affiliated rare-disease clinics are also tracked. UniteRare's specialist directory is updated continuously as new evidence becomes available.

See full Primary non-essential cutis verticis gyrata page for complete clinical details, sources, and verified-specialist listings.

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