Last update: April 2024
Also known as: cutaneous leukocytoclastic vasculitis, cutaneous leukocytoclastic angiitis, hypersensitivity angiitis and cutaneous necrotizing venulitis
What is cutaneous small vessel vasculitis?
Cutaneous small vessel vasculitis (CSVV) is a condition caused by inflammation of the skin’s blood vessels. It classically presents as areas of red or purple discoloration. This is due to bleeding under the skin and does not disappear when pressure is applied (purpura).
Who gets cutaneous small vessel vasculitis?
CSVV occurs in all age groups but is more common in the adult population. Children are more likely to be diagnosed with Henoch-Schönlein purpura (HSP), a subtype of CSVV caused by deposition of immunoglobulin A (IgA) within the blood vessel wall. HSP is often preceded by viral respiratory illness.
What causes cutaneous small vessel vasculitis?
CSVV may be caused by a reaction to medications, food and food additives, underlying infection, autoimmune connective tissue disease, malignancy or other diseases. However, in most cases there is no known underlying cause.
In a minority of affected individuals, cutaneous vasculitis can be part of a more severe vasculitis affecting other organs in the body known as “systemic vasculitis”.
What does cutaneous small vessel vasculitis look like?
The most common presentation of CSVV is raised red-purple spots (purpura, between 0.3-1 cm in diameter) and/or petechiae (purpuric lesions <3 mm in diameter) which do not disappear with pressure (i.e. non-blanching).
These lesions can join together, become ulcerated, and may be associated with blood filled blisters. The rash of vasculitis is most common on the lower legs, areas of tight-fitting clothing and areas of trauma (Koebner phenomenon). However, any surface can be involved.
Individuals affected with CSVV may experience itching, burning or pain or they may not experience any symptoms at all.
General symptoms such as fevers, weight loss and muscle aches may occur. Between 5-25% of individuals with CSVV can experience joint, genitourinary or gastrointestinal symptoms. 1 This raises the possibility of internal involvement.
In HSP, lesions tend to occur on the buttocks and lower limbs. Children may develop systemic disease with gastrointestinal, joint, and/or kidney involvement. In adults with HSP, arthritis and kidney disease occur more frequently.
How is cutaneous small vessel vasculitis diagnosed?
A detailed medical history and physical examination is essential, as well as selected laboratory studies (e.g., blood and urine tests) are essential to look for underlying causes or organ involvement. A skin biopsy is often needed to confirm the diagnosis, but it can still be difficult to distinguish between benign cases and lymphoma-associated cases. Two samples of skin are taken –one for histology (microscopic examination of the skin) and the second for immunofluorescence (to look for antibodies in the vessel walls).
How is cutaneous small vessel vasculitis treated?
Treatment options will vary depending on the individual and their needs.
General measures and symptomatic treatments may include:
- Rest and leg elevation
- Avoiding tight clothing
- Pain management with nonsteroidal anti-inflammatory drugs (NSAIDs)
- Oral antihistamines and topical corticosteroids to help relieve itching
Systemic therapy to arrest the disease process is usually reserved for those individuals who have persistent symptoms or develop skin or systemic complications, and may include:
- Systemic corticosteroids (e.g. prednisone)
- Colchicine
- Dapsone
- Hydroxychloroquine
- Systemic immunosuppressive therapy such as azathioprine, methotrexate and mycophenolate mofetil, cyclosporine, cyclophosphamide, rituximab and intravenous immunoglobulin.
It is important to rule out internal organ involvement and a referral to a physician may be necessary in those with systemic signs or symptoms. Any identified trigger or cause must also be removed or treated.
What is the likely outcome of cutaneous small vessel vasculitis?
In most individuals, the condition is self-limiting which means that it resolves spontaneously, usually within 2 to 4 weeks. Sometimes the skin inflammation can leave residual darkening of affected areas known as post-inflammatory hyperpigmentation. This usually fades slowly over many months.
Chronic or recurrent disease occurs in about 10% of affected individuals. 2