Overview
Cutaneous small vessel vasculitis (CSVV) is a condition where the small blood vessels in the skin become inflamed and damaged. It is also known as hypersensitivity vasculitis, leukocytoclastic vasculitis, or cutaneous leukocytoclastic angiitis. This inflammation is usually triggered by an immune system reaction — often in response to an infection, a medication, or another underlying illness. In some cases, no clear trigger is found. The most recognizable sign of CSVV is a rash called palpable purpura — small, raised, reddish-purple spots on the skin, most often appearing on the lower legs. These spots do not fade when you press on them, which helps doctors tell them apart from ordinary bruises or rashes. The rash can be itchy or painful, and in more severe cases, the skin may blister or develop sores. Most people with CSVV have a mild, self-limiting illness that clears up on its own or with simple treatment, especially once any triggering cause is removed. However, some people experience repeated flares or develop involvement of organs beyond the skin, such as the kidneys or joints. Treatment focuses on identifying and removing the trigger, managing symptoms, and in more persistent cases, using anti-inflammatory or immune-suppressing medications.
Also known as:
Key symptoms:
Small raised reddish-purple spots on the skin (palpable purpura), most often on the lower legsSkin rash that does not fade when pressedItching or burning sensation in the rash areaSkin blisters or open sores in severe casesJoint pain or swellingMild feverFatigue or feeling generally unwellSwelling in the lower legs or feetStomach pain or discomfort (if internal vessels are affected)Blood in the urine (if kidneys are involved)
Clinical phenotype terms (14)— hover any for plain English
Multifactorial
Caused by a mix of several genes and environmental factors
Variable
Can begin at different ages, from infancy through adulthood
Treatments
1 availableIMPAVIDO
treatment of cutaneous leishmaniasis caused by Leishmania braziliensis
Clinical Trials
View all trials with filters →No actively recruiting trials found for Cutaneous small vessel vasculitis 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
Financial Resources
1 resourcesTravel Grants
No travel grants are currently matched to Cutaneous small vessel vasculitis.
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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
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Social Security Disability
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Questions for your doctor
Bring these to your next appointment
- Q1.What do you think triggered my vasculitis, and how can we find out for sure?,Do I need any tests to check if my kidneys or other organs are affected?,How long is my rash likely to last, and what are the chances it will come back?,What medications are you recommending, and what are the main side effects I should watch for?,Are there any activities, foods, or medications I should avoid to prevent a flare?,When should I go to the emergency room versus calling your office?,Should I see any other specialists, such as a rheumatologist or kidney doctor?
Common questions about Cutaneous small vessel vasculitis
What is Cutaneous small vessel vasculitis?
Cutaneous small vessel vasculitis (CSVV) is a condition where the small blood vessels in the skin become inflamed and damaged. It is also known as hypersensitivity vasculitis, leukocytoclastic vasculitis, or cutaneous leukocytoclastic angiitis. This inflammation is usually triggered by an immune system reaction — often in response to an infection, a medication, or another underlying illness. In some cases, no clear trigger is found. The most recognizable sign of CSVV is a rash called palpable purpura — small, raised, reddish-purple spots on the skin, most often appearing on the lower legs. Th
How is Cutaneous small vessel vasculitis inherited?
Cutaneous small vessel vasculitis follows a multifactorial inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
Which specialists treat Cutaneous small vessel vasculitis?
4 specialists and care centers treating Cutaneous small vessel vasculitis are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.