Summary about Disease
Urticarial vasculitis (UV) is a skin condition characterized by hives (urticaria) accompanied by inflammation of small blood vessels in the skin (vasculitis). Unlike ordinary hives, UV lesions tend to last longer, are often painful, and may leave bruising or discoloration as they resolve. It can be a sign of an underlying systemic condition, but in some cases, the cause is unknown.
Symptoms
Hives (wheals) that are:
Longer lasting than typical hives (more than 24 hours, often 2-3 days or longer)
Painful or burning, rather than itchy (although itching can occur)
Fixed in location (don't move around like typical hives)
Leave behind a bruise-like discoloration (post-inflammatory hyperpigmentation)
Angioedema (swelling, particularly around the eyes, lips, or tongue)
Systemic symptoms (depending on the underlying cause):
Fever
Joint pain (arthralgia) or arthritis
Abdominal pain
Fatigue
Kidney problems
Lung problems
Causes
The exact cause of urticarial vasculitis is often unknown (idiopathic). However, potential causes and associations include:
Autoimmune diseases: Systemic lupus erythematosus (SLE), Sjögren's syndrome, rheumatoid arthritis
Infections: Hepatitis B or C, HIV
Medications: Certain antibiotics, NSAIDs
Cryoglobulinemia
Cancer (rarely)
Idiopathic: No identifiable underlying cause.
Medicine Used
Treatment focuses on controlling the inflammation and addressing any underlying conditions. Medications may include:
Antihistamines: To relieve itching (less effective than in typical hives)
Corticosteroids: Prednisone or other oral corticosteroids to reduce inflammation (used for short-term flares due to side effect profile)
Dapsone: An antibiotic with anti-inflammatory properties.
Colchicine: An anti-inflammatory medication.
Hydroxychloroquine: An anti-malarial drug with immunomodulatory effects.
Immunosuppressants: Azathioprine, methotrexate, cyclosporine, or mycophenolate mofetil for more severe or refractory cases.
Biologic agents: Anti-TNF agents (e.g., etanercept, infliximab) or anti-IL-1 agents (e.g., anakinra) for severe cases unresponsive to other treatments.
Treatment of underlying condition: If UV is secondary to an underlying condition, treatment of that condition is essential.
Is Communicable
Urticarial vasculitis is not communicable or contagious. It is not caused by an infectious agent that can be spread from person to person.
Precautions
Avoid known triggers (if identified).
Protect skin from sun exposure, as some forms of vasculitis can be exacerbated by sunlight.
Follow your doctor's instructions regarding medication and monitoring.
Report any new or worsening symptoms to your doctor promptly.
Maintain a healthy lifestyle with a balanced diet and regular exercise to support overall immune function.
How long does an outbreak last?
Individual lesions of urticarial vasculitis last longer than typical hives, usually more than 24 hours and often 2-3 days or longer. The overall duration of an "outbreak" or flare-up can vary widely, from weeks to months or even years, depending on the underlying cause, severity, and response to treatment. Some individuals may experience chronic, relapsing symptoms.
How is it diagnosed?
Diagnosis typically involves:
Physical examination: Assessment of the skin lesions and any associated symptoms.
Medical history: Review of your symptoms, medical history, medications, and family history.
Skin biopsy: A small sample of skin from an affected area is examined under a microscope to confirm the presence of vasculitis. Direct immunofluorescence may also be performed on the biopsy.
Blood tests: To look for signs of inflammation, autoimmune diseases, infections, and kidney or liver problems. Common tests include:
Complete blood count (CBC)
Erythrocyte sedimentation rate (ESR) or C-reactive protein (CRP)
Antinuclear antibody (ANA)
Complement levels (C3, C4, CH50)
Hepatitis B and C serologies
HIV testing
Cryoglobulins
Urinalysis: To assess kidney function.
Further investigations: May be needed to identify an underlying cause, depending on the clinical presentation and initial test results. These might include:
Rheumatoid factor (RF)
Anti-Ro/SSA and Anti-La/SSB antibodies
Kidney function tests
Chest X-ray or CT scan
Timeline of Symptoms
The timeline of symptoms can vary. Typically:
Initial presentation: Appearance of hives that are painful or burning, last longer than 24 hours, and may leave bruising.
Progression: The hives may come and go, with new lesions appearing over time. Systemic symptoms (fever, joint pain, abdominal pain) may develop concurrently or later.
Resolution: With appropriate treatment, the inflammation can be controlled, and the frequency and severity of outbreaks can be reduced. However, relapses are common. Post-inflammatory hyperpigmentation may take weeks to months to fade.
Chronic urticarial vasculitis: In some cases, symptoms may persist for months or years, requiring ongoing management.
Important Considerations
Urticarial vasculitis can be a sign of a serious underlying medical condition.
Early diagnosis and treatment are important to prevent complications and improve long-term outcomes.
It's crucial to work with a healthcare provider experienced in managing vasculitis, such as a dermatologist or rheumatologist.
Treatment is often individualized based on the severity of the condition and the presence of any underlying causes.
Regular follow-up appointments are necessary to monitor your condition and adjust treatment as needed.