Overview
Hereditary pediatric Behçet-like disease is a rare inherited condition that causes the immune system to become overactive, leading to widespread inflammation throughout the body. It shares many features with classic Behçet's disease — a well-known inflammatory condition — but it starts in childhood and is caused by changes (mutations) in specific genes rather than the complex mix of genetic and environmental factors seen in classic Behçet's. Because of this distinction, it is sometimes called 'monogenic Behçet-like disease' or 'pediatric autoinflammatory Behçet-like syndrome.' The disease mainly affects the lining of the mouth and gut, the skin, the eyes, and the joints. Children with this condition often experience painful sores in the mouth and digestive tract, skin rashes, eye inflammation, and recurring fevers. The immune system attacks the body's own tissues, causing repeated flares of symptoms that can be unpredictable. Note: This condition is listed as 'obsolete' in the Orphanet rare disease database, meaning it has been reclassified or merged into more specific diagnoses — such as ANCA-associated vasculitis or specific gene-defined autoinflammatory syndromes — as genetic understanding has improved. Treatment typically focuses on calming the immune system using anti-inflammatory and immunosuppressive medications.
Also known as:
Key symptoms:
Painful sores (ulcers) inside the mouthSores or ulcers in the digestive tract (gut)Skin rashes or soresEye inflammation (uveitis), which can cause redness, pain, or blurred visionRecurring fevers without an obvious infectionJoint pain and swellingAbdominal (belly) painDiarrhea, sometimes with bloodFatigue and low energySwollen lymph nodes
Variable
Can be inherited in different ways depending on the underlying gene
Childhood
Begins in childhood, roughly ages 1 to 12
Treatments
No FDA-approved treatments are currently listed for OBSOLETE: Hereditary pediatric Behçet-like disease.
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Specialists
View all specialists →No specialists are currently listed for OBSOLETE: Hereditary pediatric Behçet-like disease.
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 OBSOLETE: Hereditary pediatric Behçet-like disease.
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.Which specific gene mutation does my child have, and how does that affect treatment choices?,What are the early warning signs of a flare, and when should I bring my child to the emergency room?,How often should my child have eye exams, even when feeling well?,Are there clinical trials or specialized centers for this condition that we should consider?,What vaccinations are safe for my child while on immunosuppressive therapy?,How will this condition affect my child's growth and development long-term?,What support resources are available for our family?
Common questions about OBSOLETE: Hereditary pediatric Behçet-like disease
What is OBSOLETE: Hereditary pediatric Behçet-like disease?
Hereditary pediatric Behçet-like disease is a rare inherited condition that causes the immune system to become overactive, leading to widespread inflammation throughout the body. It shares many features with classic Behçet's disease — a well-known inflammatory condition — but it starts in childhood and is caused by changes (mutations) in specific genes rather than the complex mix of genetic and environmental factors seen in classic Behçet's. Because of this distinction, it is sometimes called 'monogenic Behçet-like disease' or 'pediatric autoinflammatory Behçet-like syndrome.' The disease mai
At what age does OBSOLETE: Hereditary pediatric Behçet-like disease typically begin?
Typical onset of OBSOLETE: Hereditary pediatric Behçet-like disease is childhood. Age of onset can vary across affected individuals.