Scleredema

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Overview

Scleredema, also known as scleredema adultorum or scleredema of Buschke, is a rare skin condition where the skin becomes thickened, hardened, and tight. It mainly affects the upper back, neck, and shoulders, though it can sometimes spread to the face, arms, and chest. The skin in affected areas feels woody or dough-like and may look slightly swollen or reddened. Unlike scleroderma, scleredema does not usually affect the fingers or hands. There are three main types of scleredema. Type 1 occurs after an infection, often a streptococcal throat infection, and usually resolves on its own within months to a couple of years. Type 2 is associated with a blood disorder called monoclonal gammopathy and tends to be more persistent. Type 3, the most common form, is linked to diabetes mellitus, especially poorly controlled type 2 diabetes, and tends to be chronic and long-lasting. The exact cause of scleredema is not fully understood, but it involves an abnormal buildup of a substance called mucin and collagen in the deeper layers of the skin. Treatment depends on the underlying type and severity. Options include managing the underlying condition (such as better blood sugar control in diabetes), phototherapy (UVA1 light treatment), physical therapy, and sometimes medications like methotrexate or cyclosporine. There is currently no single cure that works for all patients, and treatment results can vary widely.

Also known as:

Key symptoms:

Thickened, hardened skin on the upper back and neckTight or woody feeling of the skinDifficulty moving the neck or shoulders due to skin stiffnessSwollen or puffy appearance of affected skinReddened or darkened skin in affected areasReduced range of motion in the arms or shouldersSkin that does not pit when pressed (non-pitting swelling)Skin changes spreading to the face, chest, or armsDifficulty opening the mouth fully if the face is involvedOccasional pain or discomfort in affected areas

Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Adult

Begins in adulthood (age 18 or older)

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Scleredema.

View clinical trials →

No actively recruiting trials found for Scleredema at this time.

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

Search ClinicalTrials.gov ↗Join the Scleredema community →

No specialists are currently listed for Scleredema.

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 Scleredema.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Scleredema

No recent news articles for Scleredema.

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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.Which type of scleredema do I have, and what is causing it?,Should I be tested for diabetes or a blood disorder?,What treatment options are available for my specific type?,Is UVA1 phototherapy available and appropriate for me?,How can I maintain my range of motion and prevent further stiffness?,Will this condition get worse over time, or could it resolve on its own?,Are there any clinical trials or new treatments I should know about?

Common questions about Scleredema

What is Scleredema?

Scleredema, also known as scleredema adultorum or scleredema of Buschke, is a rare skin condition where the skin becomes thickened, hardened, and tight. It mainly affects the upper back, neck, and shoulders, though it can sometimes spread to the face, arms, and chest. The skin in affected areas feels woody or dough-like and may look slightly swollen or reddened. Unlike scleroderma, scleredema does not usually affect the fingers or hands. There are three main types of scleredema. Type 1 occurs after an infection, often a streptococcal throat infection, and usually resolves on its own within mo

How is Scleredema inherited?

Scleredema follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Scleredema typically begin?

Typical onset of Scleredema is adult. Age of onset can vary across affected individuals.