Cutaneous Vasculitis Information for Patients

What is cutaneous vasculitis?

 Vasculitis is defined as inflammation of blood vessel walls.  This inflammation causes damage to the vessel wall with subsequent leakage of red blood cells out of the vessels and into the surrounding skin.  There are various sizes of blood vessels in the body, from large to small.  In most cases when there is skin involvement, small blood vessels known as post-capillary venules are affected; this is called small vessel vasculitis.  This can lead to the development of purpura, or bruises, which are often raised or palpable.  Depending on the amount and type of inflammation and vessel wall damage, blisters, ulcers, pustules, target-like lesions, or hive-like lesions can also develop.

When medium sized blood vessels are involved, the above changes may be seen in addition to lace or net-like purpura/bruising, lace or net-like redness and patchy discoloration (livedo reticularis), ulcers, nodules in the deeper layers of the skin, or necrotic or dead tissue.  This is known as medium vessel vasculitis.

Vasculitis of the skin most commonly affects the lower legs, but can also affect the buttock area and rarely spread to the upper body.  Lesions may be itchy, painful, or have a burning sensation.

What caused my cutaneous vasculitis?

In almost half of cases of small vessel vasculitis of the skin, a specific cause is never identified (called idiopathic).  Otherwise, vasculitis usually occurs as a reaction to something else going on in the body.  In about 15-20% of cases, an infection is the cause.  Examples include bacterial infections such as Streptococcus and viral infections such as an upper respiratory tract infection or hepatitis B and C.  Another 15-20% of patients may have vasculitis as a consequence of some other condition in the body that causes excessive inflammation.  Examples include inflammatory disorders such as inflammatory bowel disease and autoimmune conditions such as rheumatoid arthritis, systemic lupus erythematosus, and Sjogren’s syndrome.  In 10-15% of cases, medications such as antibiotics and NSAIDs (non-steroidal anti-inflammatory drugs) are the cause of the small vessel vasculitis.  Rarely, the vasculitis can occur as a reaction to an underlying cancer in the body.

Causes of combined vasculitis of small and medium sized blood vessels include cryoglobulinemia and ANCA (anti-neutrophil cytoplasmic antibody)-positive diseases.  In cryoglobulinemia, the antibodies that help us fight infections collect together in colder temperatures and then attack our blood vessels which results in vasculitis; this can occur as a result of infections such as hepatitis C, autoimmune conditions such as those listed above, and lymphomas (cancer of the lymphatic system).  ANCA-positive vasculitis is an autoimmune condition of unclear cause, although it can rarely be associated with certain medications.

Cutaneous polyarteritis nodosa (PAN) is a vasculitis of medium sized blood vessels.  Similar to small vessel vasculitis of the skin, there are multiple potential causes as listed above.

What other problems am I at risk for?

Patients with cutaneous vasculitis may experience fevers, fatigue, and joint pains.  If blood vessels in organs besides the skin are affected, there may be further manifestations.  These include kidney damage such as protein and blood in the urine, gut involvement resulting in abdominal pain and bloody stools, nerve involvement causing numbness and tingling in the hands and feet, weakness, respiratory tract involvement resulting in trouble breathing including asthma-like symptoms, chest pain, cough, coughing up blood, and sinus congestion.

What should I expect?

The skin spots of cutaneous small vessel vasculitis usually come up as a crops and then resolve over a few weeks to months.  About 10% of patients will have recurrent disease showing up as crops of purpura every few months or years.  If there is a distinct cause for the vasculitis and it is addressed, there is less likely to be a recurrence.  For example, if an infection caused the vasculitis and the infection resolves or is treated, the vasculitis will also resolve.

Generally, patients with cutaneous small vessel vasculitis do well, but the severity of the disease depends on the cause.  Those with involvement of organs besides the skin and with vasculitis of medium sized vessels have a greater risk of long term consequences and a low but present risk of death.

When you are evaluated for vasculitis, your dermatologist may do a skin biopsy to evaluate the type of inflammation present and size of the vessel that is involved.  They may also do a second type of biopsy called a direct immunofluorescence (DIF) that helps to determine what type of antibodies have collected around your blood vessels.  In the majority of cases, however, a dermatologist will make the diagnosis without a skin biopsy.

Next, the doctor may try and figure out why you have a vasculitis.  They will ask you about how you feel including symptoms of infection, autoimmune conditions, inflammatory conditions, and cancer.  They will ask you about your medication list.  This information may be helpful in finding out the cause.
Also, they will assess for the involvement of the vasculitis in other organs besides your skin.  For this they will ask you relevant questions about how you feel.  If you do have involvement of other organs, you will be referred to other specialists to assist in taking care of you.

Depending on your skin examination, medical history, and a review of your symptoms, your physician may order specific labs and tests in order to get more information about your condition.  At a minimum, these labs will likely include a complete blood count, comprehensive metabolic panel, and urine test.

What can I do to make my skin better?  What will make my skin worse?

The treatment of cutaneous vasculitis starts by addressing the underlying cause.  Any suspect medications should be discontinued and if an infection, inflammatory condition, or cancer is suspected, it must be managed by the appropriate doctor.

Initial treatment recommendations include leg elevation when resting, minimizing standing for long periods of time, and the avoidance of tight fitting clothing.  Trauma can induce lesions therefore caution should be exercised.  Topical corticosteroids may be helpful and for itching, oral antihistamines can be used.  Often, the above measures alone will be of great benefit.

If the vasculitis still persists or progresses, your doctor may prescribe several weeks of oral corticosteroids such as prednisone or other oral medications such as colchicine and dapsone.  Other medications options include hydroxychloroquine, azathioprine, mycophenolate mofetil, and methotrexate.  Less commonly used, but helpful treatments when the above measures are not enough include intravenous immunoglobulin (IVIG), rituximab, cyclophosphamide, and cyclosporine.  All of these treatments require you to be closely monitored by your physician.

Is my family at risk?

Cutaneous vasculitis does not run in families.  However, if a patient has an autoimmune or inflammatory disease as the cause of the vasculitis, other family members have a slightly increased risk of developing an autoimmune condition as well, but not specifically the vasculitis.

Other resources

E-medicine article on vasculitis

Fiorentino DF. Cutaneous vasculitis. J Am Acad Dermatol. 2003;48(3):311-40.

Goeser MR, Laniosz V, Wetter DA. A practical approach to the diagnosis, evaluation, and management of cutaneous small-vessel vasculitis. Am J Clin Dermatol. 2014;15(4):299-306.

Support groups

Vasculitis Foundation