Nevus comedonicus syndrome

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ORPHA:64754OMIM:617025Q82.5
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8Treatment centers

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Overview

Nevus comedonicus syndrome (also called comedo nevus syndrome) is a rare skin condition that causes a distinctive type of birthmark called a nevus comedonicus. This birthmark looks like a cluster of enlarged, plugged pores or blackhead-like spots on the skin. These marks are caused by abnormal development of hair follicles, which become blocked and form dark, pitted lesions. The skin changes are usually present at birth or appear in early childhood, and they can occur anywhere on the body, though the face, neck, and trunk are most commonly affected. Beyond the skin, some people with this syndrome also have problems in other parts of the body. These can include eye abnormalities (such as cataracts or problems with the retina), skeletal issues (like scoliosis or extra fingers and toes), and in some cases, brain or nervous system involvement. Not everyone has all of these features — the condition can look very different from person to person. Treatment focuses on managing symptoms rather than curing the condition. Skin care, including keeping the affected areas clean and sometimes using retinoid creams, can help reduce blockages and inflammation. Surgical removal of the nevus is an option for some patients. Other symptoms, such as eye or bone problems, are treated by the relevant specialists. Regular monitoring by a team of doctors is important to catch and manage any complications early.

Key symptoms:

Clusters of enlarged, blackhead-like pores on the skin (nevus comedonicus)Dark, pitted skin lesions that may appear at birth or in early childhoodSkin inflammation or acne-like breakouts in the affected areaCysts or abscesses forming under the skin near the lesionsEye problems such as cataracts or retinal abnormalitiesScoliosis (curved spine) or other bone and joint problemsExtra fingers or toes (polydactyly)Seizures or other neurological symptoms in some casesIntellectual disability in rare casesAsymmetric distribution of skin lesions following lines on the body (Blaschko lines)

Clinical phenotype terms (16)— hover any for plain English
HamartomaHP:0010566ComedoHP:0025249Nevus flammeusHP:0001052Preaxial polydactylyHP:0100258
Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Variable

Can begin at different ages, from infancy through adulthood

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Nevus comedonicus syndrome.

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No actively recruiting trials found for Nevus comedonicus syndrome at this time.

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No specialists are currently listed for Nevus comedonicus syndrome.

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 Nevus comedonicus syndrome.

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Community

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Latest news about Nevus comedonicus syndrome

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Caregiver Resources

NORD Caregiver Resources

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Mental Health Support

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Family & Caregiver Grants

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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.Which body systems should be checked for complications related to this syndrome?,What skin care routine do you recommend, and are there specific products I should avoid?,Is genetic testing recommended for me or my family members?,What are the warning signs that the skin lesions are becoming infected, and when should I seek urgent care?,Are there any clinical trials or research studies I could participate in?,How often should I have follow-up appointments, and with which specialists?,Are there support groups or patient communities for people with nevus comedonicus syndrome?

Common questions about Nevus comedonicus syndrome

What is Nevus comedonicus syndrome?

Nevus comedonicus syndrome (also called comedo nevus syndrome) is a rare skin condition that causes a distinctive type of birthmark called a nevus comedonicus. This birthmark looks like a cluster of enlarged, plugged pores or blackhead-like spots on the skin. These marks are caused by abnormal development of hair follicles, which become blocked and form dark, pitted lesions. The skin changes are usually present at birth or appear in early childhood, and they can occur anywhere on the body, though the face, neck, and trunk are most commonly affected. Beyond the skin, some people with this synd

How is Nevus comedonicus syndrome inherited?

Nevus comedonicus syndrome follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.