Cutaneous vasculitis

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 means inflammation of blood vessels; these may be arteries, veins or both. It 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 investigations may be required for a full diagnosis.

What causes cutaneous vasculitis?

In about half of cases, no cause is found. Cutaneous vasculitis may be a result of  infection, medications, autoimmune diseases, malignancy (cancer) or blood disorders. These triggers can increase the activity of the immune system (the body's defence system) and cause it to react to the blood vessels in a way that causes damage to their walls. This, in turn, allows blood and fluid to leak from damaged blood vessels into the surrounding tissue causing a rash and swelling (cutaneous vasculitis).

Infections:

Vasculitis can occur 7-10 days after a viral or bacterial illness.

Autoimmune conditions:

Vasculitis may occur in patients who already have autoimmune diseases such as rheumatoid arthritis, lupus and inflammatory bowel disease (IBD).

Medications:

More than 100 drugs have been associated with vasculitis. These include antibiotics, anticonvulsants, diuretics and non-steroidal anti-inflammatory agents. Any new medication may be considered by the doctor as a cause of a vasculitic rash.

Malignancy or Haematological (blood) disorders:

Cancers may be associated with increased thickening of the blood or the 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 does cutaneous vasculitis look and feel like?

The most common type of cutaneous vasculitis is ‘cutaneous small vessel vasculitis’ (CSVV). CSVV can look like marks on the skin that do not fade when pressed. They can be flat, raised, or look like bruises. These marks are typically small, but may measure between 1 millimetre to several centimetres. Rarely, there may be pustules (pus-filled blister), blisters and wheals which heal leaving patches of darker coloured skin. These patches/marks often cause no symptoms, but can sometimes cause pain, burning or itching. The ankles and lower legs as well as pressure points on the knees, back of foot and lower legs are most frequently affected.

If medium-sized vessels in the skin are affected, a net-like (or lace-like) skin discolouration, ulceration (open sores) or larger raised bumps may occur.

Other symptoms to be aware of, which may suggest involvement of organs other than the skin include:

  • Fever
  • Nausea and vomiting
  • Blood in urine
  • Joint and muscle aches
  • Muscle weakness
  • Tingling or numbness in the hands and/or feet
  • Chest pain
  • Breathlessness or cough
  • Abdominal pain
  • 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 usually confirms the diagnosis in classic cases, but this is not always required. A skin biopsy is when a small sample of skin is taken to be examined under a microscope.
  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, if medication is suspected as the cause for vasculitis, this should be stopped (but only after advice by a doctor) and underlying infection should be treated.

If other systems of the body are affected, specialist input may be required in managing those systems. For example, a kidney specialist (nephrologist) would be involved if kidneys are also affected.

Otherwise, initial treatment measures include:

  • Leg elevation
  • Adequate rest
  • Analgesics (pain killers)
  • Antihistamines
  • 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 medications may be required such as oral corticosteroids, colchicine, dapsone and treatments that control the immune system.

 Can cutaneous vasculitis be cured?

The outlook is good with full recovery seen in the majority 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 appear darker compared to unaffected skin. This discolouration usually takes months to fade back to the normal colour.

The outlook for systemic vasculitis (i.e. vasculitis affecting internal organs) is dependent on the severity of other organs involved.

Where can I get more information about vasculitis?

Web links to patient support groups:

https://www.vasculitis.org.uk/

Web links to detailed leaflets:

http://www.dermnetnz.org/topics/cutaneous-vasculitis/

https://www.pcds.org.uk/clinical-guidance/vasculitis-and-capillaritis

Jargon Buster: https://www.skinhealthinfo.org.uk/support-resources/jargon-buster/

Please note that the BAD provides web links to additional resources to help people access a range of information about their treatment or skin condition. The views expressed in these external resources may not be shared by the BAD or its members. The BAD has no control of and does not endorse the content of external links.

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, JULY 2023

NEXT REVIEW DATE | JULY 2026

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