Kindler epidermolysis bullosa

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ORPHA:2908OMIM:173650Q81.8
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16Specialists8Treatment centers

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Overview

Kindler epidermolysis bullosa (also called Kindler syndrome or Kindler-Weary syndrome) is a rare inherited skin disorder that causes the skin to blister very easily, even from minor friction or injury. It belongs to a group of conditions called epidermolysis bullosa (EB), but it has some unique features that set it apart from other types. The condition is caused by changes (mutations) in a gene called FERMT1, which provides instructions for making a protein called kindlin-1. This protein helps hold the layers of skin together. Without it working properly, the skin becomes extremely fragile. From birth or early infancy, affected individuals develop painful blisters on the skin, especially on the hands and feet. Over time, the skin may become thin and wrinkled in appearance, a feature called poikiloderma. Sun sensitivity is also common. As people age, the condition can affect the inside of the body too, including the mouth, gut, and urinary tract, causing scarring and narrowing of these passages. There is currently no cure for Kindler epidermolysis bullosa. Treatment focuses on protecting the skin, managing wounds carefully, preventing infections, and treating complications as they arise. A team of specialists is usually needed to manage the many ways this condition can affect the body throughout a person's life.

Also known as:

Key symptoms:

Skin blistering from minor bumps or friction, especially on hands and feetBlistering present from birth or early infancySkin that becomes thin, wrinkled, and mottled over time (poikiloderma)Increased sensitivity to sunlightScarring of the skin after blisters healWebbing or fusion of fingers or toes over timePainful sores inside the mouthDifficulty swallowing due to scarring in the throat or esophagusNarrowing of the urethra causing problems with urinationInflammation and scarring in the gutIncreased risk of skin cancer (squamous cell carcinoma) in adulthoodFragile gums and dental problemsChronic wounds that are slow to heal

Clinical phenotype terms (41)— hover any for plain English
PeriodontitisHP:0000704PoikilodermaHP:0001029Laryngeal stenosisHP:0001602PhimosisHP:0001741
Inheritance

Autosomal recessive

Passed on when both parents carry the same gene change; often skips generations

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Kindler epidermolysis bullosa.

View clinical trials →

No actively recruiting trials found for Kindler epidermolysis bullosa at this time.

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

Search ClinicalTrials.gov ↗Join the Kindler epidermolysis bullosa community →

Specialists

16 foundView all specialists →
SK
Susanne Krämer
Specialist
3 Kindler epidermolysis bullosa publications
AK
Alfred Klausegger
Specialist
2 Kindler epidermolysis bullosa publications
AM
Alexandra DJ Mancini, MSc
GREENACRES, FL
Specialist
PI on 1 active trial
YW
Yao Wang
Specialist
1 Kindler epidermolysis bullosa publication
MB
Mihail-Alexandru Badea
Specialist
1 Kindler epidermolysis bullosa publication
JB
Jose Ignacio Barrios
Specialist
1 Kindler epidermolysis bullosa publication
SD
Sorina Danescu
Specialist
1 Kindler epidermolysis bullosa publication
DK
Demet Kartal
Specialist
1 Kindler epidermolysis bullosa publication
IY
Ivelina Yordanova
Specialist
1 Kindler epidermolysis bullosa publication
KL
Kaiyang Li
Specialist
1 Kindler epidermolysis bullosa publication
CL
Chaocheng Liu
Specialist
1 Kindler epidermolysis bullosa publication
AH
Anna Lena Hillebrecht
HENDERSON, IL
Specialist
1 Kindler epidermolysis bullosa publication
IF
Ignacia Fuentes
Specialist
2 Kindler epidermolysis bullosa publications
AB
Agnes Bloch-Zupan
Specialist
2 Kindler epidermolysis bullosa publications
RS
Reinhard Schilke
Specialist
1 Kindler epidermolysis bullosa publication
EL
Enrique Ponce de León
Specialist
1 Kindler epidermolysis bullosa publication

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 Kindler epidermolysis bullosa.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Kindler epidermolysis bullosa

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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.What type of wound dressings are best for my specific situation, and where can I get them?,How often should I be screened for skin cancer, and what should I watch for at home?,Are there any clinical trials or new treatments I should know about for Kindler epidermolysis bullosa?,What signs of internal complications, like esophageal or urinary tract narrowing, should I watch for?,Should other family members be tested to see if they are carriers of the FERMT1 mutation?,What pain management options are available to make daily wound care more comfortable?,Are there specialized EB centers or multidisciplinary clinics you can refer me to?

Common questions about Kindler epidermolysis bullosa

What is Kindler epidermolysis bullosa?

Kindler epidermolysis bullosa (also called Kindler syndrome or Kindler-Weary syndrome) is a rare inherited skin disorder that causes the skin to blister very easily, even from minor friction or injury. It belongs to a group of conditions called epidermolysis bullosa (EB), but it has some unique features that set it apart from other types. The condition is caused by changes (mutations) in a gene called FERMT1, which provides instructions for making a protein called kindlin-1. This protein helps hold the layers of skin together. Without it working properly, the skin becomes extremely fragile. F

How is Kindler epidermolysis bullosa inherited?

Kindler epidermolysis bullosa follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Kindler epidermolysis bullosa typically begin?

Typical onset of Kindler epidermolysis bullosa is neonatal. Age of onset can vary across affected individuals.

Which specialists treat Kindler epidermolysis bullosa?

16 specialists and care centers treating Kindler epidermolysis bullosa are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.