Overview
Hereditary acrokeratotic poikiloderma of Kindler-Weary, now more commonly known as Kindler syndrome (or Kindler epidermolysis bullosa), is a rare inherited skin disorder. The term in this Orphanet entry is marked as obsolete because the condition has been reclassified under the broader name Kindler syndrome. This disease affects the skin in several ways. From birth or early infancy, affected individuals develop skin blistering, especially on the hands and feet. Over time, the skin develops poikiloderma, which means it shows a mix of thinning, changes in color (both lighter and darker patches), and visible small blood vessels. The skin on the hands and feet may also become thickened (a feature called acrokeratosis). Sun sensitivity is a hallmark feature, meaning the skin burns or blisters more easily with sun exposure. As patients age, the blistering tends to improve, but the skin changes, thinning, and sensitivity persist. The condition can also affect the lining of the mouth, esophagus, and other mucosal surfaces, sometimes causing difficulty eating or urinary problems. There is currently no cure for Kindler syndrome. Treatment focuses on protecting the skin from trauma and sun exposure, managing blisters and wounds, and monitoring for complications including an increased risk of skin cancer (squamous cell carcinoma) later in life.
Key symptoms:
Skin blistering, especially on hands and feet in infancySkin thinning (atrophy) that worsens with agePatchy skin color changes (lighter and darker areas)Visible tiny blood vessels on the skinThickened skin on palms and solesIncreased sensitivity to sunlightFragile skin that tears or bruises easilyGum disease and gum shrinkageMouth sores or blistering inside the mouthDifficulty swallowing due to esophageal narrowingWebbing or fusion of fingers or toesUrinary tract problemsIncreased risk of skin cancer in adulthoodDry or wrinkled-looking skin
Autosomal recessive
Passed on when both parents carry the same gene change; often skips generations
Neonatal
Begins at or shortly after birth (first 4 weeks)
Treatments
No FDA-approved treatments are currently listed for OBSOLETE: Hereditary acrokeratotic poikiloderma of Kindler-Weary.
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Specialists
View all specialists →No specialists are currently listed for OBSOLETE: Hereditary acrokeratotic poikiloderma of Kindler-Weary.
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to OBSOLETE: Hereditary acrokeratotic poikiloderma of Kindler-Weary.
Community
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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.How can I best protect my child's skin from blistering and sun damage?,How often should skin cancer screening be done, and when should it start?,What dental care routine do you recommend to prevent gum disease?,Are there any clinical trials or new treatments being studied for Kindler syndrome?,What signs of complications should I watch for at home?,Should other family members be tested for the FERMT1 gene mutation?,Can you refer us to a multidisciplinary team experienced with this condition?
Common questions about OBSOLETE: Hereditary acrokeratotic poikiloderma of Kindler-Weary
What is OBSOLETE: Hereditary acrokeratotic poikiloderma of Kindler-Weary?
Hereditary acrokeratotic poikiloderma of Kindler-Weary, now more commonly known as Kindler syndrome (or Kindler epidermolysis bullosa), is a rare inherited skin disorder. The term in this Orphanet entry is marked as obsolete because the condition has been reclassified under the broader name Kindler syndrome. This disease affects the skin in several ways. From birth or early infancy, affected individuals develop skin blistering, especially on the hands and feet. Over time, the skin develops poikiloderma, which means it shows a mix of thinning, changes in color (both lighter and darker patches),
How is OBSOLETE: Hereditary acrokeratotic poikiloderma of Kindler-Weary inherited?
OBSOLETE: Hereditary acrokeratotic poikiloderma of Kindler-Weary follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does OBSOLETE: Hereditary acrokeratotic poikiloderma of Kindler-Weary typically begin?
Typical onset of OBSOLETE: Hereditary acrokeratotic poikiloderma of Kindler-Weary is neonatal. Age of onset can vary across affected individuals.