Overview
Huriez syndrome is a very rare inherited skin condition that affects the skin, nails, and increases the risk of certain cancers. It is also sometimes called scleroatrophic and keratotic dermatosis of the limbs, or sclerotylosis. The condition is present from birth, though it becomes more noticeable over time. The skin on the hands and feet tends to become thick, tight, and hardened — a process called scleroatrophy. The palms and soles may also develop rough, scaly patches (palmoplantar keratoderma). The fingernails and toenails are often underdeveloped, thin, or abnormally shaped from birth. One of the most important features of Huriez syndrome is a significantly increased risk of developing squamous cell carcinoma, a type of skin cancer, particularly in the areas of thickened skin. This cancer risk can appear as early as the third or fourth decade of life, making regular skin monitoring essential. Some people also experience reduced sweating in affected areas. There is currently no cure for Huriez syndrome. Treatment focuses on managing symptoms — keeping the skin moisturized, using keratolytic creams to soften thickened skin, and closely monitoring for signs of skin cancer. Early detection of cancer is critical and can significantly improve outcomes. A team of specialists including dermatologists and geneticists is important for ongoing care.
Also known as:
Key symptoms:
Tight, hardened, or thickened skin on the hands and feet (scleroatrophy)Rough, scaly skin on the palms and soles (palmoplantar keratoderma)Underdeveloped, thin, or misshapen fingernails and toenails present from birthReduced or absent sweating in affected skin areasIncreased risk of squamous cell skin cancer, especially in thickened skin areasSkin that may appear shiny or tight over the fingers and handsPossible mild redness or inflammation in affected skin areas
Clinical phenotype terms (7)— hover any for plain English
Autosomal dominant
Passed on from just one parent; each child has about a 50% chance of inheriting it
Neonatal
Begins at or shortly after birth (first 4 weeks)
Treatments
No FDA-approved treatments are currently listed for Huriez syndrome.
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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 Huriez syndrome.
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 often should I have my skin checked for signs of skin cancer, and what should I look out for at home?,What skin care products or creams do you recommend to manage the thickened skin on my hands and feet?,Should my children or other family members be tested for this condition?,Are there any clinical trials or new treatments being studied for Huriez syndrome?,What are the early warning signs of squamous cell carcinoma I should watch for in my affected skin areas?,Should I see a genetic counselor, and what can genetic testing tell me about my family's risk?,Are there any activities or exposures I should avoid to protect my skin?
Common questions about Huriez syndrome
What is Huriez syndrome?
Huriez syndrome is a very rare inherited skin condition that affects the skin, nails, and increases the risk of certain cancers. It is also sometimes called scleroatrophic and keratotic dermatosis of the limbs, or sclerotylosis. The condition is present from birth, though it becomes more noticeable over time. The skin on the hands and feet tends to become thick, tight, and hardened — a process called scleroatrophy. The palms and soles may also develop rough, scaly patches (palmoplantar keratoderma). The fingernails and toenails are often underdeveloped, thin, or abnormally shaped from birth.
How is Huriez syndrome inherited?
Huriez syndrome follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Huriez syndrome typically begin?
Typical onset of Huriez syndrome is neonatal. Age of onset can vary across affected individuals.
Which specialists treat Huriez syndrome?
1 specialists and care centers treating Huriez syndrome are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.