Hereditary neurocutaneous malformation

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ORPHA:1062OMIM:106070D18.0
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23Specialists8Treatment centers

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UniteRare data is sourced from FDA.gov, ClinicalTrials.gov, Orphanet, OMIM, and NORD.
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Overview

Hereditary neurocutaneous malformation, also known as hereditary neurocutaneous angioma or neurocutaneous hemangioma, is a very rare condition that involves abnormal blood vessel growths (vascular malformations or hemangiomas) that affect both the skin and the nervous system. The condition is present from birth or early childhood and belongs to a group of disorders sometimes called neurocutaneous syndromes or phakomatoses, which are conditions where both the skin and the nervous system develop abnormally together. Patients may have visible red or purple birthmarks or growths on the skin (hemangiomas), along with similar vascular malformations in the brain or spinal cord. Depending on the location and size of these malformations, symptoms can range from mild skin findings to more serious neurological problems such as seizures, headaches, weakness, or developmental delays. The severity varies widely from person to person, even within the same family. Because this condition is extremely rare, treatment is mainly supportive and symptom-based. Vascular malformations on the skin may be treated with laser therapy or surgery if needed. Neurological symptoms such as seizures are managed with standard medications. Regular monitoring with brain imaging is important to watch for changes in any internal vascular malformations. A team of specialists including dermatologists, neurologists, and neurosurgeons typically work together to manage care.

Key symptoms:

Red or purple birthmarks on the skin (hemangiomas)Vascular growths in the brain or spinal cordSeizures or epilepsyHeadachesMuscle weakness on one side of the bodyDevelopmental delaysVision problemsSkin discoloration or raised skin lesionsNumbness or tinglingDifficulty with balance or coordinationLearning difficulties

Clinical phenotype terms (4)— hover any for plain English
Horner syndromeHP:0002277
Inheritance

Variable

Can be inherited in different ways depending on the underlying gene

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗OMIM ↗NORD ↗

FDA & Trial Timeline

1 event
Mar 2023Mind and Skin - the Neurocutaneous Axis in Atopic Eczema

King's College London

TrialRECRUITING

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Treatments

No FDA-approved treatments are currently listed for Hereditary neurocutaneous malformation.

View clinical trials →

No actively recruiting trials found for Hereditary neurocutaneous malformation at this time.

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

Search ClinicalTrials.gov ↗Join the Hereditary neurocutaneous malformation community →

Specialists

23 foundView all specialists →
BM
Brigitte C. Widemann, MD
BETHESDA, MD
Specialist
PI on 6 active trials
BM
Brigitte C Widemann, M.D.
Bethesda, Maryland
Specialist

Rare Disease Specialist

PI on 18 active trials
KK
Katarzyna Kotulska-Jozwiak
Specialist
PI on 1 active trial2 Hereditary neurocutaneous malformation publications
PM
Prashant Chittiboina, M.D.
Bethesda, Maryland
Specialist

Rare Disease Specialist

PI on 7 active trials
DM
Donald Gilbert, MD MS
Cincinnati, Ohio
Specialist

Rare Disease Specialist

PI on 1 active trial
VP
Vanessa Merker, PhD
Boston, Massachusetts
Specialist

Rare Disease Specialist

PI on 3 active trials
DA
Dr. Evdokia Anagnostou
Specialist
PI on 1 active trial
KG
Kan Gong
NEW YORK, NY
Specialist
PI on 3 active trials9 Hereditary neurocutaneous malformation publications
KP
Kan Gong, PhD
NEW YORK, NY
Specialist
PI on 1 active trial
SP
Scott Plotkin, MD, PhD
BUFFALO, NY
Specialist
PI on 3 active trials
JM
John D Heiss, M.D.
BETHESDA, MD
Specialist
PI on 6 active trials
EM
Emily Y Chew, M.D.
BETHESDA, MD
Specialist
PI on 8 active trials
SP
Staci M Peron, Ph.D.
Bethesda, Maryland
Specialist

Rare Disease Specialist

PI on 8 active trials
RM
Richard R Anderson, MD
Specialist
PI on 4 active trials
QL
Qingfeng Li
Shanghai
Specialist

Rare Disease Specialist

PI on 1 active trial572 Hereditary neurocutaneous malformation publications
EJ
Eric Jonasch
HOUSTON, TX
Specialist
PI on 3 active trials2 Hereditary neurocutaneous malformation publications
IP
Irene Franco, PhD
CORPUS CHRISTI, TX
Specialist
PI on 1 active trial
AM
Alessandro Larcher, MD
Specialist
PI on 1 active trial1 Hereditary neurocutaneous malformation publication
AP
Anna Jansen, MD, PhD
Silver Spring, Maryland
Specialist

Rare Disease Specialist

PI on 2 active trials
BP
Bartlett D. Moore, PhD
HOUSTON, TX
Specialist
PI on 1 active trial
SM
Shafali Jeste, MD
LOS ANGELES, CA
Specialist
PI on 1 active trial
AM
Angelo M Taveira-DaSilva, M.D.
BETHESDA, MD
Specialist
PI on 1 active trial

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Hereditary neurocutaneous malformation.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Hereditary neurocutaneous malformation

Disease timeline:

New recruiting trial: Selumetinib for the Prevention of Plexiform Neurofibroma Growth in NF Type 1

A new clinical trial is recruiting patients for Hereditary neurocutaneous malformation

New recruiting trial: Feasibility and Tolerability of IMLYGIC for the Treatment of Cutaneous Neurofibromas in Adults With NF1

A new clinical trial is recruiting patients for Hereditary neurocutaneous malformation

New recruiting trial: Real-World Treatment Study of Koselugo (Selumetinib)

A new clinical trial is recruiting patients for Hereditary neurocutaneous malformation

New recruiting trial: A Decentralized Clinical Trial to Promote Evidence-Based Care for Underserved Patients With Neurofibromatosis 1

A new clinical trial is recruiting patients for Hereditary neurocutaneous malformation

New recruiting trial: Mind and Skin - the Neurocutaneous Axis in Atopic Eczema

A new clinical trial is recruiting patients for Hereditary neurocutaneous malformation

New recruiting trial: A Retrospective Study on Epidemiological Characteristics of Chinese NF1 Patients in Real World (PROMISE)

A new clinical trial is recruiting patients for Hereditary neurocutaneous malformation

New recruiting trial: Glioma Developmental and HyperActive Ras Tumor (DHART) Board

A new clinical trial is recruiting patients for Hereditary neurocutaneous malformation

New recruiting trial: Development and Validation of Patient Reported Outcome (PRO) Measures for Individuals With Neurofibromatosis 1 (NF1) and Plexiform Neurofibromas (pNFs)

A new clinical trial is recruiting patients for Hereditary neurocutaneous malformation

New recruiting trial: Development of Patient-Reported Outcome Measures Assessing Tumor Visibility and Appearance Concerns in Neurofibromatosis Type 1: A Qualitative Study

A new clinical trial is recruiting patients for Hereditary neurocutaneous malformation

New recruiting trial: Clinical and Demographic Characteristics of Adult p Atients With NEurofibromatosis in RUSsia

A new clinical trial is recruiting patients for Hereditary neurocutaneous malformation

Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

Mental Health Support

Rare disease caregiving can be isolating. Connect with counseling and peer support.

Family & Caregiver Grants

Financial assistance programs specifically for caregivers of rare disease patients.

Social Security Disability

Learn how rare disease patients may qualify for SSDI/SSI benefits.

Questions for your doctor

Bring these to your next appointment

  • Q1.What is the exact location and size of the vascular malformations in my child's brain or spine?,How often should we have MRI scans to monitor for changes?,What are the warning signs that a vascular malformation is bleeding?,Are there any activity restrictions we should follow?,Should other family members be screened for this condition?,What treatment options are available if the vascular malformations grow or cause new symptoms?,Are there any clinical trials or research studies we could participate in?

Common questions about Hereditary neurocutaneous malformation

What is Hereditary neurocutaneous malformation?

Hereditary neurocutaneous malformation, also known as hereditary neurocutaneous angioma or neurocutaneous hemangioma, is a very rare condition that involves abnormal blood vessel growths (vascular malformations or hemangiomas) that affect both the skin and the nervous system. The condition is present from birth or early childhood and belongs to a group of disorders sometimes called neurocutaneous syndromes or phakomatoses, which are conditions where both the skin and the nervous system develop abnormally together. Patients may have visible red or purple birthmarks or growths on the skin (hema

At what age does Hereditary neurocutaneous malformation typically begin?

Typical onset of Hereditary neurocutaneous malformation is neonatal. Age of onset can vary across affected individuals.

Which specialists treat Hereditary neurocutaneous malformation?

23 specialists and care centers treating Hereditary neurocutaneous malformation are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.