Granulomatous slack skin

Last reviewed

🖨 Print for my doctorAdvocacy Hub →
ORPHA:33111C84.0
Who is this for?
Show terms as
1Specialists8Treatment centers

Where are you in your journey?

UniteRare data is sourced from FDA.gov, ClinicalTrials.gov, Orphanet, OMIM, and NORD.
Report missing data

Overview

Granulomatous slack skin (GSS) is an extremely rare type of skin lymphoma — a cancer that starts in white blood cells called T-cells that live in the skin. It is considered a subtype of mycosis fungoides, which is the most common form of cutaneous T-cell lymphoma. GSS has its own distinct features that set it apart from other skin lymphomas. The most striking feature of GSS is the gradual development of loose, hanging, wrinkled skin — especially in the armpits (axillae) and groin area. This happens because the cancerous T-cells destroy the elastic fibers in the skin, causing it to lose its firmness and sag. Under a microscope, doctors can see clusters of immune cells called granulomas, which is how the disease gets its name. GSS progresses very slowly over many years. Most people are diagnosed in adulthood, though it can appear earlier. Because it is so rare, treatment is not fully standardized, but options include skin-directed therapies, radiation, and systemic treatments used for other lymphomas. A notable concern is that a significant number of people with GSS also develop another lymphoma called Hodgkin lymphoma, so ongoing monitoring is very important.

Key symptoms:

Loose, sagging, wrinkled skin especially in the armpits and groinSlow-growing skin plaques or patches that become pendulous over timeSkin that hangs in folds due to loss of elasticityItching of affected skin areasRedness or discoloration of the skin in affected areasGradual enlargement of affected skin areas over yearsPossible swollen lymph nodesFatigue in some cases

Clinical phenotype terms (10)— hover any for plain English
Hodgkin lymphomaHP:0012189Stiff skinHP:0030053Abnormal lymph node morphologyHP:0002733
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 Granulomatous slack skin.

View clinical trials →

No actively recruiting trials found for Granulomatous slack skin at this time.

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

Search ClinicalTrials.gov ↗Join the Granulomatous slack skin community →

Specialists

1 foundView all specialists →
BP
Brian Poligone, MD, PhD
FAIRPORT, NY
Specialist
PI on 1 active trial

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 Granulomatous slack skin.

Search all travel grants →NORD Financial Assistance ↗

Community

Open Granulomatous slack skinForum →

No community posts yet. Be the first to share your experience with Granulomatous slack skin.

Start the conversation →

Latest news about Granulomatous slack skin

No recent news articles for Granulomatous slack skin.

Follow this condition to be notified when news becomes available.

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 advanced is my disease, and do I need treatment right now or can we monitor it first?,What is my risk of developing Hodgkin lymphoma or another type of lymphoma, and how will you monitor for this?,Which treatment option do you recommend for me, and what are the side effects?,Will the sagging skin ever improve, and are there any options to address it?,How often do I need follow-up appointments and what tests will be done at each visit?,Should I see a specialist at a center that has experience with cutaneous lymphomas?,Are there any clinical trials I might be eligible for?

Common questions about Granulomatous slack skin

What is Granulomatous slack skin?

Granulomatous slack skin (GSS) is an extremely rare type of skin lymphoma — a cancer that starts in white blood cells called T-cells that live in the skin. It is considered a subtype of mycosis fungoides, which is the most common form of cutaneous T-cell lymphoma. GSS has its own distinct features that set it apart from other skin lymphomas. The most striking feature of GSS is the gradual development of loose, hanging, wrinkled skin — especially in the armpits (axillae) and groin area. This happens because the cancerous T-cells destroy the elastic fibers in the skin, causing it to lose its fi

How is Granulomatous slack skin inherited?

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

At what age does Granulomatous slack skin typically begin?

Typical onset of Granulomatous slack skin is adult. Age of onset can vary across affected individuals.

Which specialists treat Granulomatous slack skin?

1 specialists and care centers treating Granulomatous slack skin are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.