Interstitial granulomatous dermatitis with arthritis

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Overview

Interstitial granulomatous dermatitis with arthritis (IGDA) is a rare inflammatory skin and joint condition. It is sometimes called 'rope sign' disease because one of its most recognizable features is a cord-like thickening of skin that can be felt under the surface, especially on the sides of the trunk. The skin develops firm, rope-like bands or folds, along with reddish or skin-colored bumps and plaques that may be itchy or tender. These skin changes most often appear on the trunk, inner thighs, and armpits. Along with the skin symptoms, most people with IGDA also experience joint pain, swelling, and stiffness — similar to what is seen in rheumatoid arthritis or lupus. In fact, IGDA is strongly linked to underlying autoimmune diseases, meaning the immune system is mistakenly attacking the body's own tissues. Many people diagnosed with IGDA are later found to have a related autoimmune condition such as rheumatoid arthritis, lupus, or another connective tissue disease. Treatment focuses on managing symptoms and addressing the underlying autoimmune condition. Doctors often use anti-inflammatory medications, corticosteroids, and disease-modifying drugs. The skin findings may improve when the underlying autoimmune disease is treated effectively. While IGDA is not life-threatening on its own, it can significantly affect quality of life and comfort.

Also known as:

Key symptoms:

Firm, rope-like bands or cords felt under the skin, especially on the sides of the trunkRed, pink, or skin-colored bumps and raised patches on the skinItchy or tender skin lesionsSkin folds or plaques on the inner thighs and armpitsJoint pain and swellingJoint stiffness, especially in the morningFatigueSkin that feels thickened or hardened in affected areasOccasional skin discoloration or darkening in affected areas

Clinical phenotype terms (8)— hover any for plain English
Rheumatoid arthritisHP:0001370Rheumatoid factor positiveHP:0002923
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 Interstitial granulomatous dermatitis with arthritis.

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No actively recruiting trials found for Interstitial granulomatous dermatitis with arthritis at this time.

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No specialists are currently listed for Interstitial granulomatous dermatitis with arthritis.

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 Interstitial granulomatous dermatitis with arthritis.

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Community

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Questions for your doctor

Bring these to your next appointment

  • Q1.Do I need to be tested for an underlying autoimmune disease like rheumatoid arthritis or lupus?,What treatment do you recommend for my skin symptoms and joint pain, and how long will it take to see results?,What are the risks and side effects of the medications you are recommending?,How often should I have follow-up appointments and blood tests?,Are there any lifestyle changes or things I should avoid that could help manage my symptoms?,What signs should prompt me to contact you or seek urgent care?,Are there any clinical trials or new treatments I should know about?

Common questions about Interstitial granulomatous dermatitis with arthritis

What is Interstitial granulomatous dermatitis with arthritis?

Interstitial granulomatous dermatitis with arthritis (IGDA) is a rare inflammatory skin and joint condition. It is sometimes called 'rope sign' disease because one of its most recognizable features is a cord-like thickening of skin that can be felt under the surface, especially on the sides of the trunk. The skin develops firm, rope-like bands or folds, along with reddish or skin-colored bumps and plaques that may be itchy or tender. These skin changes most often appear on the trunk, inner thighs, and armpits. Along with the skin symptoms, most people with IGDA also experience joint pain, swe

How is Interstitial granulomatous dermatitis with arthritis inherited?

Interstitial granulomatous dermatitis with arthritis follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Interstitial granulomatous dermatitis with arthritis typically begin?

Typical onset of Interstitial granulomatous dermatitis with arthritis is adult. Age of onset can vary across affected individuals.