What are the aims of this leaflet?
This leaflet is written to help you understand more about cutaneous vasculitis. It tells you what it is, what causes it, what can be done about it and where to get more information about this condition.
What is cutaneous vasculitis?
Vasculitis is a term referring to inflammation of blood vessels; these may be arteries, veins or both, and can affect any part of the body. When vasculitis affects small or medium sized blood vessels in the skin, it is known as cutaneous vasculitis. Occasionally cutaneous vasculitis can be a sign of inflammation occurring in other organs (a systemic vasculitis) and further investigation may be required for a full diagnosis.
What causes cutaneous vasculitis?
Cutaneous vasculitis may be caused by an infection, medications, autoimmune diseases, malignancy (cancer) or blood disorders. In about half of cases, no cause is found. The triggers can induce increased activity of the immune system (the body’s defence system), with increased production of inflammatory mediators which cause damage to blood vessel walls. Fluid may also leak from the blood vessels into the surrounding tissues which can result in swelling especially of the lower legs.
Vasculitis can occur 7-10 days after a viral or bacterial illness.
Vasculitis may occur in patients with autoimmune diseases (where the immune system reacts against patients own tissues) such as rheumatoid arthritis, lupus and inflammatory bowel disease.
More than 100 drugs have been associated in drug-induced vasculitis. These include antibiotics, anticonvulsants, diuretics and non-steroidal anti-inflammatory agents. Any new medication taken will be the initial suspect of causing a vasculitic rash.
Malignancy or Haematological (blood) disorders:
Cancers may be associated with increased thickening of the blood or development of antibodies which can cause blood vessel damage. Abnormalities of the red or white blood cells can also lead to vasculitis.
Is vasculitis hereditary?
No, vasculitis is not known to be hereditary.
What are the features of cutaneous vasculitis?
The most common type of cutaneous vasculitis is cutaneous small vessel vasculitis (CSVV). This usually appears as non-blanching (the colour does not disappear when pressed) or bruise like marks on the skin, some of which may be raised lumps. These marks are typically small, but may measure between 1 millimetre to several centimetres as rarely, pustules, blisters and wheals which heal with darker skin colouration. These often cause no symptoms but can sometimes cause pain, burning and itching. The ankles and lower legs as well as pressure points on the knees, back of foot and lower legs are most frequently affected. Pressure points are found near joints and junctures and are specific sensitive areas on the body.
If medium-sized vessels in the skin are affected, a network-like skin discolouration, ulceration or larger raised lesions may be seen.
Other symptoms to be aware of, which may suggest involvement of organs other than the skin include:
Nausea and vomiting
Blood in urine
Joint and muscle aches
Tingling or numbness in the hands and/or feet
Breathlessness or cough
Blood in the faeces
How is cutaneous vasculitis diagnosed?
The diagnosis can often be made based on the appearance of the skin.
1. A skin biopsy (tissue sample) usually confirms the diagnosis in classic cases, but this is not always required.
2. Blood tests are often carried out to check for the presence of infection, autoimmune and inflammatory conditions as well as blood abnormalities.
3. A urine sample, blood pressure check and blood tests are useful to exclude involvement of other organs.
4. Very rarely, scans may be needed to check for vasculitis in other organs.
How can cutaneous vasculitis be treated?
Any underlying causes should be dealt with. For example, any possible medication reactions should be stopped (but only after advice by a doctor) and underlying infection should be treated.
Otherwise, initial treatment measures include:
Non-steroidal anti-inflammatory drugs (except if there is kidney involvement)
Steroid creams and ointments applied to the skin
Dressings may be required if the skin is ulcerated
If the episode of vasculitis is prolonged, severe or leading to ulceration, oral medication such as those listed below may be required:
In patients with severe or systemic disease treatment with other medication which suppress or modulate change to adjust the immune system, including immunotherapy may be required.
Can cutaneous vasculitis be cured?
The outlook is good with a full recovery in 90% of cases (providing only the skin is involved). The rash may recur at intervals for some time after the initial episode. Even after successful treatment, the areas affected by vasculitis can become more pigmented (brown or black) compared to non-affected skin. This discolouration usually takes months to fade to normal.
Where can I get more information about vasculitis?
Web links to detailed leaflets:
Links to patient support groups:
For details of source materials used please contact the Clinical Standards Unit (firstname.lastname@example.org).
This leaflet aims to provide accurate information about the subject and is a consensus of the views held by representatives of the British Association of Dermatologists: individual patient circumstances may differ, which might alter both the advice and course of therapy given to you by your doctor.
This leaflet has been assessed for readability by the British Association of Dermatologists’ Patient Information Lay Review Panel
BRITISH ASSOCIATION OF DERMATOLOGISTS
PATIENT INFORMATION LEAFLET
PRODUCED NOVEMBER 2013
UPDATED MARCH 2017
REVIEW DATE MARCH 2020