Overview
Schnitzler syndrome is a rare condition where the immune system becomes overactive, causing a chronic skin rash and other symptoms throughout the body. It is sometimes called 'Schnitzler's syndrome' after the French dermatologist Liliane Schnitzler, who first described it in 1972. The condition belongs to a group of diseases called autoinflammatory disorders, meaning the immune system triggers inflammation without a clear infection or allergy causing it. The most noticeable sign is a recurring hive-like rash (urticaria) that usually does not itch much. Along with the rash, most people develop a protein called a monoclonal immunoglobulin (usually IgM, sometimes IgG) in their blood — this is a key feature doctors look for when making the diagnosis. People also commonly experience fever, joint and bone pain, swollen lymph nodes, and fatigue. The good news is that effective treatments exist. The drug anakinra (Kineret), which blocks a specific inflammatory signal called interleukin-1, has shown remarkable results and is considered the best current treatment. Most people respond very well and can achieve near-complete relief of symptoms. However, long-term monitoring is important because a small number of people with Schnitzler syndrome may develop a blood cancer called lymphoma over time.
Key symptoms:
Chronic hive-like skin rash (urticaria) that comes and goesRecurrent fever episodesJoint pain and swellingBone pain, especially in the legs and lower backFatigue and feeling generally unwellSwollen lymph nodesEnlarged spleen or liverAbnormal protein (monoclonal immunoglobulin) found in blood testsWeight loss in some casesElevated inflammatory markers on blood tests
Clinical phenotype terms (21)— hover any for plain English
Sporadic
Usually appears on its own, not inherited from a parent
Adult
Begins in adulthood (age 18 or older)
Treatments
No FDA-approved treatments are currently listed for Schnitzler syndrome.
View clinical trials →Clinical Trials
View all trials with filters →No actively recruiting trials found for Schnitzler syndrome at this time.
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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 Schnitzler 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
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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.Is anakinra the right treatment for me, and how quickly should I expect it to work?,How often do I need blood tests and check-ups to monitor for lymphoma?,What warning signs should make me seek emergency care?,Are there any clinical trials I could join for Schnitzler syndrome?,Will my insurance cover anakinra or canakinumab, and what are my options if it does not?,Should I see a hematologist regularly even if I feel well on treatment?,Are there any lifestyle changes or things I should avoid that could trigger flares?
Common questions about Schnitzler syndrome
What is Schnitzler syndrome?
Schnitzler syndrome is a rare condition where the immune system becomes overactive, causing a chronic skin rash and other symptoms throughout the body. It is sometimes called 'Schnitzler's syndrome' after the French dermatologist Liliane Schnitzler, who first described it in 1972. The condition belongs to a group of diseases called autoinflammatory disorders, meaning the immune system triggers inflammation without a clear infection or allergy causing it. The most noticeable sign is a recurring hive-like rash (urticaria) that usually does not itch much. Along with the rash, most people develop
How is Schnitzler syndrome inherited?
Schnitzler syndrome follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Schnitzler syndrome typically begin?
Typical onset of Schnitzler syndrome is adult. Age of onset can vary across affected individuals.
Which specialists treat Schnitzler syndrome?
20 specialists and care centers treating Schnitzler syndrome are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.