Parana hard skin syndrome

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ORPHA:2812OMIM:260530L91.8
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Overview

Parana hard skin syndrome is an extremely rare genetic condition first described in families from the Parana state of Brazil. It is also sometimes called 'stiff skin syndrome, Parana type' or 'Parana hard skin disease.' This condition mainly affects the skin and connective tissues, causing the skin to become abnormally thick, hard, and tight. The hardening of the skin can limit movement of the joints and make it difficult to bend or straighten the arms, legs, and fingers. People with this condition may also experience growth problems and short stature. The disease typically becomes apparent in early childhood, when parents may notice that the child's skin feels unusually firm or that the child has difficulty moving certain joints. Over time, the skin thickening can progress and may affect large areas of the body. Some individuals may also develop changes in facial features due to the tightened skin. Because this condition is so rare, there is no specific cure or targeted treatment available. Management focuses on supportive care, including physical therapy to maintain joint mobility, skin care, and monitoring for complications. Doctors may also use medications to help manage symptoms such as pain or inflammation. Research into the underlying genetic cause is ongoing, and understanding the exact mechanism may eventually lead to more targeted therapies.

Also known as:

Key symptoms:

Thick, hard skinTight skin that limits movementJoint stiffness and limited range of motionDifficulty bending fingers, arms, or legsShort stature or growth delayChanges in facial appearance due to tight skinReduced flexibilitySkin that feels woody or leathery to the touchDifficulty with fine motor tasksMuscle weakness or wasting in some cases

Clinical phenotype terms (12)— hover any for plain English
Restricted chest movementHP:0006596Generalized hyperpigmentationHP:0007440
Inheritance

Autosomal recessive

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

Age of Onset

Childhood

Begins in childhood, roughly ages 1 to 12

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Parana hard skin syndrome.

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No actively recruiting trials found for Parana hard skin syndrome at this time.

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No specialists are currently listed for Parana hard skin syndrome.

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 Parana hard skin syndrome.

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Community

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Latest news about Parana hard skin syndrome

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Caregiver Resources

NORD Caregiver Resources

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

Mental Health Support

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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 we slow down the progression of skin hardening and joint stiffness?,What type and frequency of physical therapy is recommended?,Are there any new research studies or clinical trials for this condition?,Should we pursue genetic testing for our family, and what would it involve?,What signs should we watch for that would require urgent medical attention?,Are there assistive devices or adaptive tools that could help with daily activities?,Can you refer us to a genetic counselor to discuss family planning and recurrence risk?

Common questions about Parana hard skin syndrome

What is Parana hard skin syndrome?

Parana hard skin syndrome is an extremely rare genetic condition first described in families from the Parana state of Brazil. It is also sometimes called 'stiff skin syndrome, Parana type' or 'Parana hard skin disease.' This condition mainly affects the skin and connective tissues, causing the skin to become abnormally thick, hard, and tight. The hardening of the skin can limit movement of the joints and make it difficult to bend or straighten the arms, legs, and fingers. People with this condition may also experience growth problems and short stature. The disease typically becomes apparent i

How is Parana hard skin syndrome inherited?

Parana hard skin syndrome follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Parana hard skin syndrome typically begin?

Typical onset of Parana hard skin syndrome is childhood. Age of onset can vary across affected individuals.