OBSOLETE: Hereditary epidermolysis bullosa associated with ocular features

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Overview

Hereditary epidermolysis bullosa (EB) associated with ocular features is a rare genetic skin condition that has been reclassified and is now considered obsolete as a standalone diagnosis in some medical databases. This means that the condition has been reorganized under other, more specific forms of epidermolysis bullosa. Epidermolysis bullosa refers to a group of inherited disorders where the skin is extremely fragile and blisters easily from minor friction, rubbing, or even without any obvious cause. In some forms of EB, the eyes can also be affected, leading to problems such as corneal erosions (painful scratches on the surface of the eye), scarring of the eye surface, and vision difficulties. Patients with these forms of EB typically develop skin blistering at birth or in early infancy. The blisters can occur anywhere on the body, including inside the mouth and on the hands and feet. When the eyes are involved, patients may experience recurring eye pain, tearing, sensitivity to light, and blurred vision. Over time, repeated eye injuries can lead to scarring that may permanently affect sight. Treatment is mainly supportive and focuses on wound care, preventing infections, protecting the skin from trauma, and managing eye complications with the help of an ophthalmologist. There is currently no cure for any form of epidermolysis bullosa, though research into gene therapy and other advanced treatments is ongoing. Early and coordinated care from a team of specialists can significantly improve quality of life.

Key symptoms:

Fragile skin that blisters easilySkin blisters from minor rubbing or frictionOpen sores and wounds on the skinCorneal erosions (painful scratches on the eye surface)Eye pain and tearingSensitivity to lightBlurred vision or vision lossScarring of the skinScarring on the eye surfaceBlisters inside the mouthDifficulty eating or swallowingNail abnormalities or nail lossItching or burning sensation on the skinSkin infections from open wounds

Inheritance

Variable

Can be inherited in different ways depending on the underlying gene

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for OBSOLETE: Hereditary epidermolysis bullosa associated with ocular features.

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No actively recruiting trials found for OBSOLETE: Hereditary epidermolysis bullosa associated with ocular features at this time.

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No specialists are currently listed for OBSOLETE: Hereditary epidermolysis bullosa associated with ocular features.

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 OBSOLETE: Hereditary epidermolysis bullosa associated with ocular features.

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Community

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Caregiver Resources

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

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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 specific subtype of epidermolysis bullosa does my child or I have, and what gene is involved?,How often should we see an ophthalmologist to monitor for eye complications?,What is the best wound care routine to prevent infections and minimize scarring?,Are there any clinical trials or new treatments we should consider?,What signs of infection or eye emergency should prompt us to seek immediate care?,How can we manage pain effectively on a daily basis?,Are there genetic counseling services available to help us understand the risk for future children?

Common questions about OBSOLETE: Hereditary epidermolysis bullosa associated with ocular features

What is OBSOLETE: Hereditary epidermolysis bullosa associated with ocular features?

Hereditary epidermolysis bullosa (EB) associated with ocular features is a rare genetic skin condition that has been reclassified and is now considered obsolete as a standalone diagnosis in some medical databases. This means that the condition has been reorganized under other, more specific forms of epidermolysis bullosa. Epidermolysis bullosa refers to a group of inherited disorders where the skin is extremely fragile and blisters easily from minor friction, rubbing, or even without any obvious cause. In some forms of EB, the eyes can also be affected, leading to problems such as corneal eros

At what age does OBSOLETE: Hereditary epidermolysis bullosa associated with ocular features typically begin?

Typical onset of OBSOLETE: Hereditary epidermolysis bullosa associated with ocular features is neonatal. Age of onset can vary across affected individuals.