Linear and whorled nevoid hypermelanosis

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ORPHA:79150OMIM:614323L81.4
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8Treatment centers

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Overview

Linear and whorled nevoid hypermelanosis (LWNH) is a rare skin condition where patches of darker skin appear in distinctive streaky or swirling patterns across the body. These patterns follow invisible lines on the skin called Blaschko's lines, which trace the paths that skin cells travel during early development in the womb. The condition is also sometimes called 'linear and whorled nevoid hyperpigmentation' or referred to by the abbreviation LWNH. The darker skin patches are usually present at birth or appear within the first few months of life. They are not raised, do not cause itching or pain, and are not contagious. The patches tend to become more noticeable during the first two years of life and may fade slightly in adulthood. The color difference is caused by extra melanin, the natural pigment that gives skin its color, being deposited in certain skin cells. While LWNH is primarily a skin condition, some people — particularly those with more widespread pigmentation — may also have neurological differences such as intellectual disability, seizures, or developmental delays. Treatment focuses on managing any associated symptoms, as the skin pigmentation itself does not require medical treatment. Sun protection is generally recommended to prevent the patches from darkening further.

Also known as:

Key symptoms:

Streaky or swirling patches of darker skin following curved lines on the bodySkin patches present at birth or appearing in early infancyPatches that do not cause itching, pain, or raised textureDarker skin areas most commonly on the trunk, arms, and legsPatches that may become more visible in the first two years of lifePossible intellectual disability in some casesPossible seizures or epilepsy in some affected individualsPossible developmental delays in motor or speech skillsPatches that may fade slightly with ageNo blistering or skin breakdown associated with the patches

Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Linear and whorled nevoid hypermelanosis.

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No actively recruiting trials found for Linear and whorled nevoid hypermelanosis at this time.

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No specialists are currently listed for Linear and whorled nevoid hypermelanosis.

View NORD Rare Disease Centers ↗Undiagnosed Disease Network ↗

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 Linear and whorled nevoid hypermelanosis.

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Community

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Caregiver Resources

NORD Caregiver Resources

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

Mental Health Support

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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.Does my child need any genetic testing to understand the cause of their skin pigmentation?,Should we do any neurological tests, such as an EEG or brain MRI, given the skin findings?,How should we protect the affected skin from the sun, and are there any products you recommend?,Are there any signs I should watch for that would suggest the condition is affecting the nervous system?,Will the skin patches change or fade as my child gets older?,Are there any support groups or patient communities for families affected by LWNH?,Should other family members be evaluated, or is this condition unlikely to affect them?

Common questions about Linear and whorled nevoid hypermelanosis

What is Linear and whorled nevoid hypermelanosis?

Linear and whorled nevoid hypermelanosis (LWNH) is a rare skin condition where patches of darker skin appear in distinctive streaky or swirling patterns across the body. These patterns follow invisible lines on the skin called Blaschko's lines, which trace the paths that skin cells travel during early development in the womb. The condition is also sometimes called 'linear and whorled nevoid hyperpigmentation' or referred to by the abbreviation LWNH. The darker skin patches are usually present at birth or appear within the first few months of life. They are not raised, do not cause itching or

How is Linear and whorled nevoid hypermelanosis inherited?

Linear and whorled nevoid hypermelanosis follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Linear and whorled nevoid hypermelanosis typically begin?

Typical onset of Linear and whorled nevoid hypermelanosis is neonatal. Age of onset can vary across affected individuals.