Idiopathic peliosis hepatis

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Overview

Idiopathic peliosis hepatis is a rare liver condition where unusual blood-filled cavities, or cysts, form inside the liver tissue. The word 'peliosis' comes from the Greek word for 'bruise,' which describes the purplish appearance of these blood-filled spaces. 'Idiopathic' means that no clear underlying cause can be found — in other cases, peliosis hepatis can be linked to certain medications, infections like bartonella, or other diseases, but in the idiopathic form, the trigger remains unknown. These blood-filled cavities can range from tiny spots to larger pockets spread throughout the liver. In many people, the condition causes no symptoms at all and is discovered by accident during imaging done for another reason. When symptoms do occur, they may include abdominal pain, an enlarged liver, nausea, or signs of liver dysfunction. The most serious risk is spontaneous rupture of one of these cavities, which can cause life-threatening internal bleeding. Treatment depends on how severe the condition is. Mild cases may only need monitoring. More serious cases may require stopping any suspected triggering medications, treating underlying infections, or in rare situations, surgery or liver transplantation. Because this is a rare and poorly understood condition, management is largely individualized and guided by specialist experience.

Also known as:

Key symptoms:

Pain or discomfort in the upper right abdomenEnlarged liver (hepatomegaly)Nausea or vomitingUnexplained weight lossFatigue and general weaknessYellowing of the skin or eyes (jaundice)Abnormal liver blood test resultsFeeling of fullness or bloating in the bellyFever in some casesSudden severe abdominal pain if a cyst ruptures

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 Idiopathic peliosis hepatis.

View clinical trials →

No actively recruiting trials found for Idiopathic peliosis hepatis at this time.

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

Search ClinicalTrials.gov ↗Join the Idiopathic peliosis hepatis community →

No specialists are currently listed for Idiopathic peliosis hepatis.

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 Idiopathic peliosis hepatis.

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Community

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Latest news about Idiopathic peliosis hepatis

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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.What is causing my peliosis hepatis, and have all possible triggers been ruled out?,How often do I need imaging or blood tests to monitor my liver?,What symptoms should prompt me to go to the emergency room immediately?,Are there any medications, supplements, or foods I should avoid?,What is the risk that my condition will worsen, and what would that look like?,Should I be referred to a liver transplant center for evaluation, even as a precaution?,Are there any clinical trials or research studies I could participate in?

Common questions about Idiopathic peliosis hepatis

What is Idiopathic peliosis hepatis?

Idiopathic peliosis hepatis is a rare liver condition where unusual blood-filled cavities, or cysts, form inside the liver tissue. The word 'peliosis' comes from the Greek word for 'bruise,' which describes the purplish appearance of these blood-filled spaces. 'Idiopathic' means that no clear underlying cause can be found — in other cases, peliosis hepatis can be linked to certain medications, infections like bartonella, or other diseases, but in the idiopathic form, the trigger remains unknown. These blood-filled cavities can range from tiny spots to larger pockets spread throughout the live

How is Idiopathic peliosis hepatis inherited?

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

At what age does Idiopathic peliosis hepatis typically begin?

Typical onset of Idiopathic peliosis hepatis is adult. Age of onset can vary across affected individuals.