Becker nevus syndrome

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ORPHA:64755OMIM:604919D22.5
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Overview

Becker nevus syndrome (also called Becker melanosis syndrome or pigmented hairy epidermal nevus syndrome) is a rare condition that combines a distinctive skin birthmark called a Becker nevus with other physical differences affecting the muscles, bones, and skin. A Becker nevus is a large, flat, brownish patch of skin that often develops during childhood or the teenage years, most commonly on the shoulder, chest, or upper back. Over time, the patch may become darker and grow coarse, dark hair. In Becker nevus syndrome, this skin finding is accompanied by additional features on the same side of the body, such as one breast being smaller than the other, differences in arm or shoulder size, or underdeveloped chest muscles. The condition is not life-threatening, but it can affect a person's appearance and self-confidence. The skin patch itself is benign, meaning it is not cancerous. However, the associated physical differences — like uneven breast development or muscle underdevelopment — can sometimes cause emotional distress or physical discomfort. The syndrome is more commonly noticed in males, partly because the skin changes are more visible and the hormonal triggers (like testosterone) seem to play a role in how the nevus develops. There is no cure for Becker nevus syndrome, but several treatments can help manage the appearance of the skin patch and address related physical differences. Laser therapy can reduce the pigmentation and hair growth in the nevus. Surgical options may be considered for significant physical asymmetry, such as breast reconstruction. A team of specialists — including dermatologists, plastic surgeons, and orthopedic doctors — can work together to create a personalized care plan.

Also known as:

Key symptoms:

Large, flat, brownish skin patch (Becker nevus) usually on the shoulder, chest, or upper backCoarse, dark hair growing within the skin patchOne breast being noticeably smaller than the other (breast asymmetry)Underdeveloped chest muscle (hypoplasia of the pectoralis major muscle) on the same side as the nevusDifferences in arm or shoulder size between the two sides of the bodyDifferences in nipple size or positionMild differences in rib or spine shape on the affected sideSkin within the nevus may feel slightly thicker or raised over timeAcne-like skin changes within the nevus area in some people

Clinical phenotype terms (19)— hover any for plain English
Abnormal scrotum morphologyHP:0000045Hypoplastic labia minoraHP:0000064Rib fusionHP:0000902Supernumerary nippleHP:0002558Shoulder girdle muscle atrophyHP:0003724Supernumerary ribsHP:0005815Aplasia/Hypoplasia of the breastsHP:0010311HamartomaHP:0010566Upper limb asymmetryHP:0100560
Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Juvenile

Begins in the teen years

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Becker nevus syndrome.

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No actively recruiting trials found for Becker nevus syndrome at this time.

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Specialists

1 foundView all specialists →

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 Becker nevus syndrome.

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Community

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Latest news about Becker nevus syndrome

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Caregiver Resources

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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.What physical differences should I watch for as my child grows, and how often should we have check-ups?,Is laser treatment a good option for reducing the appearance of the skin patch, and what results can we realistically expect?,Are there any risks of the skin patch becoming cancerous over time?,What surgical options are available if the physical asymmetry (such as uneven breast or muscle size) becomes a significant concern?,Should my child see a psychologist or counselor to help with body image and self-esteem?,Are there any support groups or patient communities for people with Becker nevus syndrome?,Does this condition run in families, and is there any genetic testing that would be helpful?

Common questions about Becker nevus syndrome

What is Becker nevus syndrome?

Becker nevus syndrome (also called Becker melanosis syndrome or pigmented hairy epidermal nevus syndrome) is a rare condition that combines a distinctive skin birthmark called a Becker nevus with other physical differences affecting the muscles, bones, and skin. A Becker nevus is a large, flat, brownish patch of skin that often develops during childhood or the teenage years, most commonly on the shoulder, chest, or upper back. Over time, the patch may become darker and grow coarse, dark hair. In Becker nevus syndrome, this skin finding is accompanied by additional features on the same side of

How is Becker nevus syndrome inherited?

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

At what age does Becker nevus syndrome typically begin?

Typical onset of Becker nevus syndrome is juvenile. Age of onset can vary across affected individuals.

Which specialists treat Becker nevus syndrome?

1 specialists and care centers treating Becker nevus syndrome are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.