Overview
Cutaneous polyarteritis nodosa (CPAN), also called cutaneous PAN or benign cutaneous periarteritis nodosa, is a rare inflammatory disease that affects the small and medium-sized blood vessels (arteries) in the skin. Unlike the more serious systemic form of polyarteritis nodosa, which can damage organs throughout the body, cutaneous PAN is mainly limited to the skin and sometimes nearby tissues like muscles and joints. However, it can still cause significant discomfort and affect quality of life. The hallmark symptoms include painful nodules (firm lumps) under the skin, most commonly on the lower legs. These nodules may be red, purple, or skin-colored. Over time, the skin may develop a net-like, mottled pattern called livedo reticularis, which looks like a lace-like purplish discoloration. Some patients also experience skin ulcers, joint pain, muscle aches, and mild fever. In some cases, the disease has been linked to prior streptococcal infections, hepatitis B, or other triggers, though often no clear cause is found. Treatment focuses on controlling inflammation and managing symptoms. Mild cases may respond to nonsteroidal anti-inflammatory drugs (NSAIDs) or colchicine. More severe cases may require corticosteroids like prednisone or other immunosuppressive medications such as methotrexate, azathioprine, or dapsone. The disease tends to follow a chronic, relapsing course, meaning symptoms may come and go over months or years. While CPAN does not typically progress to systemic polyarteritis nodosa, long-term follow-up is important to monitor for any changes.
Also known as:
Key symptoms:
Painful lumps or nodules under the skin, especially on the lower legsNet-like, mottled purplish skin pattern (livedo reticularis)Skin ulcers that may be slow to healJoint pain and swellingMuscle aches and tendernessMild feverFatigue and general feeling of being unwellNumbness or tingling in the feet or handsSwelling of the legs or anklesTender red or purple patches on the skinSkin discoloration that worsens in cold weather
Sporadic
Usually appears on its own, not inherited from a parent
Variable
Can begin at different ages, from infancy through adulthood
FDA & Trial Timeline
2 eventsUniversity of Utah — PHASE1
University of Pennsylvania — PHASE2
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
No FDA-approved treatments are currently listed for Cutaneous polyarteritis nodosa.
1 clinical trialare actively recruiting — trials can provide access to cutting-edge therapies.
View clinical trials →Rare Disease Specialist
Treatment Centers
8 centersUniversity of Iowa
📍 Iowa City, Iowa
👤 Anthony A. Amato, MD
Huntsman Cancer Institute
📍 Salt Lake City, Utah
👤 Janssen Research & Development, LLC Clinical Trial
Stanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 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
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
Travel Grants
No travel grants are currently matched to Cutaneous polyarteritis nodosa.
Community
No community posts yet. Be the first to share your experience with Cutaneous polyarteritis nodosa.
Start the conversation →Latest news about Cutaneous polyarteritis nodosa
Disease timeline:
New recruiting trial: A Randomized Multicenter Study for Isolated Skin Vasculitis
A new clinical trial is recruiting patients for Cutaneous polyarteritis nodosa
New trial: Defactinib and Avutometinib, With or Without Encorafenib, for the Treatment of Patients With Brain M
Phase PHASE1 trial recruiting. Defactinib
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.Could my symptoms be caused by an infection like streptococcus, and should I be tested?,How can we be sure the disease is limited to my skin and has not affected my internal organs?,What treatment options are best for my level of disease, and what are the side effects?,How long will I need to take medication, and what happens if I stop?,What should I do if I notice new nodules or my skin ulcers are getting worse?,Are there lifestyle changes that can help reduce flare-ups?,How often should I have follow-up visits and blood tests to monitor my condition?
Common questions about Cutaneous polyarteritis nodosa
What is Cutaneous polyarteritis nodosa?
Cutaneous polyarteritis nodosa (CPAN), also called cutaneous PAN or benign cutaneous periarteritis nodosa, is a rare inflammatory disease that affects the small and medium-sized blood vessels (arteries) in the skin. Unlike the more serious systemic form of polyarteritis nodosa, which can damage organs throughout the body, cutaneous PAN is mainly limited to the skin and sometimes nearby tissues like muscles and joints. However, it can still cause significant discomfort and affect quality of life. The hallmark symptoms include painful nodules (firm lumps) under the skin, most commonly on the lo
How is Cutaneous polyarteritis nodosa inherited?
Cutaneous polyarteritis nodosa follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
Are there clinical trials for Cutaneous polyarteritis nodosa?
Yes — 1 recruiting clinical trial is currently listed for Cutaneous polyarteritis nodosa on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.
Which specialists treat Cutaneous polyarteritis nodosa?
3 specialists and care centers treating Cutaneous polyarteritis nodosa are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.