Overview
Single-organ polyarteritis nodosa (also called localized polyarteritis nodosa or cutaneous polyarteritis nodosa when it affects the skin) is a rare form of vasculitis — a condition where the immune system mistakenly attacks the walls of medium-sized blood vessels (arteries) in just one organ or tissue. Unlike classic polyarteritis nodosa, which can affect blood vessels throughout the body, this form stays limited to a single organ. The most commonly affected organ is the skin, but it can also involve the appendix, gallbladder, uterus, testicles, breast, or other individual organs. When the skin is involved, patients typically notice painful lumps or nodules under the skin, a net-like purplish discoloration of the skin called livedo reticularis, and sometimes skin ulcers. Some people also experience mild joint pain, muscle aches, or fatigue. When other organs are affected, symptoms depend on which organ is involved — for example, abdominal pain if the appendix or gallbladder is affected. The treatment approach depends on which organ is involved and how severe the symptoms are. For skin-limited disease, treatment often includes nonsteroidal anti-inflammatory drugs (NSAIDs), low-dose corticosteroids, or other immunosuppressive medications. In cases involving internal organs, surgical removal of the affected organ (such as appendectomy) may be both diagnostic and curative. The overall outlook is generally much better than classic polyarteritis nodosa, as the disease tends to stay localized, though skin forms can sometimes follow a relapsing course over months or years.
Also known as:
Key symptoms:
Painful lumps or nodules under the skinNet-like purplish skin discoloration (livedo reticularis)Skin ulcers that are slow to healJoint pain or swellingMuscle achesFatigue or general tirednessNumbness or tingling in the hands or feetAbdominal pain (if an internal organ is involved)Fever or feeling unwellTender areas on the legs or armsSkin color changes in cold weather
Sporadic
Usually appears on its own, not inherited from a parent
Variable
Can begin at different ages, from infancy through adulthood
Treatments
No FDA-approved treatments are currently listed for Single-organ polyarteritis nodosa.
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Specialists
View all specialists →No specialists are currently listed for Single-organ polyarteritis nodosa.
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 Single-organ polyarteritis nodosa.
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 my disease truly limited to one organ, and how can we be sure it hasn't spread?,What treatment options are best for my specific situation, and what are the side effects?,How often will I need follow-up visits and blood tests?,What are the chances that my disease will come back after treatment?,Could my condition ever progress to systemic polyarteritis nodosa?,Are there lifestyle changes that could help manage my symptoms or reduce flares?,Should I see any additional specialists for my care?
Common questions about Single-organ polyarteritis nodosa
What is Single-organ polyarteritis nodosa?
Single-organ polyarteritis nodosa (also called localized polyarteritis nodosa or cutaneous polyarteritis nodosa when it affects the skin) is a rare form of vasculitis — a condition where the immune system mistakenly attacks the walls of medium-sized blood vessels (arteries) in just one organ or tissue. Unlike classic polyarteritis nodosa, which can affect blood vessels throughout the body, this form stays limited to a single organ. The most commonly affected organ is the skin, but it can also involve the appendix, gallbladder, uterus, testicles, breast, or other individual organs. When the sk
How is Single-organ polyarteritis nodosa inherited?
Single-organ polyarteritis nodosa follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.