Ulerythema ophryogenesis

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ORPHA:3406OMIM:604093L66.4
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5Specialists8Treatment centers

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

Ulerythema ophryogenesis (also called keratosis pilaris atrophicans faciei or folliculitis ulerythematosa reticulata) is a rare inherited skin condition that mainly affects the eyebrows, cheeks, and sometimes the scalp. The name comes from Greek words meaning 'scarring redness of the eyebrows.' The condition causes the hair follicles in these areas to become inflamed, plugged with keratin (a skin protein), and eventually scarred. Over time, this scarring leads to permanent hair loss, especially in the outer parts of the eyebrows — a feature that is often one of the most noticeable signs. The skin in affected areas typically looks red, rough, and slightly bumpy, similar to 'chicken skin.' As the condition progresses, the skin can become thin and develop a pitted or net-like scar pattern. The cheeks and temples are commonly involved, and in some people the scalp is also affected. The condition usually appears in infancy or early childhood and may slowly worsen during childhood before stabilizing in adulthood. There is no cure for ulerythema ophryogenesis. Treatment focuses on managing symptoms, reducing redness and roughness, and slowing progression. Moisturizers, gentle exfoliants, and topical retinoids are commonly used. The condition is not life-threatening, but it can affect a person's appearance and self-confidence. It is sometimes associated with other syndromes, such as Noonan syndrome or cardio-facio-cutaneous syndrome.

Key symptoms:

Loss of hair in the outer parts of the eyebrowsRed, rough, bumpy skin on the cheeks and templesSmall keratin plugs blocking hair folliclesScarring of the skin around hair folliclesPitted or net-like scar pattern on affected skinThinning of the skin in affected areas over timeRedness and inflammation around hair folliclesRough 'chicken skin' texture on the facePossible involvement of the scalp with hair thinningGradual worsening during childhood that may stabilize in adulthood

Clinical phenotype terms (14)— hover any for plain English
Facial erythemaHP:0001041Sparse lateral eyebrowHP:0005338Follicular hyperkeratosisHP:0007502Keratosis pilarisHP:0032152Hyperkeratotic papuleHP:0045059Abnormal forehead morphologyHP:0000290Abnormality of the chinHP:0000306Abnormal cheek morphologyHP:0004426Contact dermatitisHP:0032282
Inheritance

Autosomal dominant

Passed on from just one parent; each child has about a 50% chance of inheriting it

Age of Onset

Infantile

Begins in infancy, roughly 1 month to 2 years old

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Ulerythema ophryogenesis.

View clinical trials →

No actively recruiting trials found for Ulerythema ophryogenesis at this time.

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Search ClinicalTrials.gov ↗Join the Ulerythema ophryogenesis community →

Specialists

5 foundView all specialists →
MM
Murad Alam, MD
CHICAGO, IL
Specialist
PI on 14 active trials
AP
Akmal Saad, professor
Specialist
PI on 1 active trial1 Ulerythema ophryogenesis publication
JM
Jeffrey Dover, MD
CHESTNUT HILL, MA
Specialist
PI on 2 active trials
MM
Myriam Gonzalez, MD
Specialist
PI on 1 active trial
SM
Sarah Hsu, 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 Ulerythema ophryogenesis.

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Community

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Latest news about Ulerythema ophryogenesis

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Caregiver Resources

NORD 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.Is my child's ulerythema ophryogenesis part of a broader genetic syndrome, and should we have genetic testing?,What skin care routine do you recommend to slow the progression of scarring?,Are there any treatments that can reduce the redness and roughness on my child's face?,Will the eyebrow hair loss get worse over time, and is there anything that can prevent it?,Are there any clinical trials or new treatments being studied for this condition?,Should other family members be evaluated, given that this condition can be inherited?,What signs should I watch for that would mean we need to come back sooner?

Common questions about Ulerythema ophryogenesis

What is Ulerythema ophryogenesis?

Ulerythema ophryogenesis (also called keratosis pilaris atrophicans faciei or folliculitis ulerythematosa reticulata) is a rare inherited skin condition that mainly affects the eyebrows, cheeks, and sometimes the scalp. The name comes from Greek words meaning 'scarring redness of the eyebrows.' The condition causes the hair follicles in these areas to become inflamed, plugged with keratin (a skin protein), and eventually scarred. Over time, this scarring leads to permanent hair loss, especially in the outer parts of the eyebrows — a feature that is often one of the most noticeable signs. The

How is Ulerythema ophryogenesis inherited?

Ulerythema ophryogenesis follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Ulerythema ophryogenesis typically begin?

Typical onset of Ulerythema ophryogenesis is infantile. Age of onset can vary across affected individuals.

Which specialists treat Ulerythema ophryogenesis?

5 specialists and care centers treating Ulerythema ophryogenesis are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.