Proliferating trichilemmal cyst

Last reviewed

🖨 Print for my doctorAdvocacy Hub →
ORPHA:492L72.1
Who is this for?
Show terms as
8Treatment centers

Where are you in your journey?

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

Overview

A proliferating trichilemmal cyst (also called a proliferating trichilemmal tumor or proliferating pilar cyst) is a rare growth that develops from the outer root sheath of a hair follicle, most often on the scalp. It starts as a slow-growing lump under the skin that can look and feel like a simple cyst at first. Over time, it may grow larger and, in some cases, can change into a more aggressive form that behaves like a skin cancer. Most of these growths are benign (not cancerous), but a small number can become malignant (cancerous), which is why doctors take them seriously. The condition most commonly affects middle-aged to older women, and the scalp is by far the most frequent location. The lump may be painless for a long time, but it can become tender, ulcerate (break open), or bleed as it grows. Because it can look similar to other skin tumors, a tissue biopsy is needed to confirm the diagnosis. Treatment usually involves surgical removal of the entire growth. When caught early and fully removed, the outlook is generally very good. However, if the tumor has developed cancerous features or has spread, more aggressive treatment such as wider surgery or radiation therapy may be needed. Regular follow-up after surgery is important to watch for any return of the growth.

Key symptoms:

A slow-growing lump or bump on the scalpFirm or rubbery texture under the skinPainless swelling that may gradually get largerSkin over the lump may become red or irritatedThe lump may open up, ulcerate, or bleedTenderness or soreness around the growthHair loss over the affected areaThe lump may feel attached to deeper tissue as it growsOccasional discharge or crusting from the surface

Clinical phenotype terms (3)— hover any for plain English
Epidermoid cystHP:0200040
Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Adult

Begins in adulthood (age 18 or older)

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Proliferating trichilemmal cyst.

View clinical trials →

No actively recruiting trials found for Proliferating trichilemmal cyst at this time.

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

Search ClinicalTrials.gov ↗Join the Proliferating trichilemmal cyst community →

No specialists are currently listed for Proliferating trichilemmal cyst.

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 Proliferating trichilemmal cyst.

Search all travel grants →NORD Financial Assistance ↗

Community

Open Proliferating trichilemmal cystForum →

No community posts yet. Be the first to share your experience with Proliferating trichilemmal cyst.

Start the conversation →

Latest news about Proliferating trichilemmal cyst

No recent news articles for Proliferating trichilemmal cyst.

Follow this condition to be notified when news becomes available.

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.Is my tumor benign, borderline, or malignant, and what does that mean for my treatment?,Were the surgical margins clear, and is there any chance the tumor was not fully removed?,How often do I need follow-up appointments, and what should I watch for between visits?,Do I need any additional treatment such as radiation after surgery?,What are the chances this tumor could come back, and what would treatment look like if it does?,Should any of my family members be checked for similar growths?,Are there any clinical trials or specialist centers I should know about for this condition?

Common questions about Proliferating trichilemmal cyst

What is Proliferating trichilemmal cyst?

A proliferating trichilemmal cyst (also called a proliferating trichilemmal tumor or proliferating pilar cyst) is a rare growth that develops from the outer root sheath of a hair follicle, most often on the scalp. It starts as a slow-growing lump under the skin that can look and feel like a simple cyst at first. Over time, it may grow larger and, in some cases, can change into a more aggressive form that behaves like a skin cancer. Most of these growths are benign (not cancerous), but a small number can become malignant (cancerous), which is why doctors take them seriously. The condition most

How is Proliferating trichilemmal cyst inherited?

Proliferating trichilemmal cyst follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Proliferating trichilemmal cyst typically begin?

Typical onset of Proliferating trichilemmal cyst is adult. Age of onset can vary across affected individuals.