Cutaneous Vasculitis

Overview

Cutaneous vasculitis is a kind of vasculitis that affects the skin and subcutaneous tissue but not the internal organs.

Any type of primary or secondary vasculitis, including serum sickness, infections (e.g., hepatitis C), malignancies, rheumatologic or other autoimmune disorders such hypersensitivity, can impact the skin.

Vasculitis can be localized or be part of a systemic primary or secondary vasculitic disorder. Livedo reticularis, purpura, petechiae, nodules, and deep ulcers are typically caused by vasculitis of deeper, medium, or large vessels. Biopsy is required for diagnosis. Treatment is influenced by the etiology and severity of the disease.

What is Cutaneous Vasculitis?

Cutaneous vasculitis is an uncommon condition that causes inflammation of the blood vessels, which can limit blood flow and damage essential organs and tissues. This disorder, formerly known as hypersensitivity vasculitis, most usually affects the skin. It usually affects people above the age of 16. Cutaneous vasculitis is also known as allergic vasculitis and leukocytoclastic vasculitis.

Cutaneous vasculitis is frequently caused by an allergic reaction to a drug or by an infection, and it normally resolves after the offending medication or infection is stopped. To manage inflammation, some patients will require corticosteroids in conjunction with immune-suppressing medicines. Most cases resolve between weeks to months if no organs are involved. However, because cutaneous vasculitis can be chronic, with exacerbations and remissions, continued medical treatment may be required.

The following terms have been used inconsistently in the literature to describe cutaneous vasculitis:

Cutaneous vasculitis: It is a kind of vasculitis that affects just the skin and not the internal organs.
Cutaneous small-vessel vasculitis (CSVV): It is a kind of vasculitis that affects the skin's small vessels but not the internal organs. CSVV is sometimes used to describe small-vessel vasculitis of unclear etiology (also called idiopathic cutaneous small-vessel vasculitis).
Leukocytoclastic vasculitis: This is a frequent kind of CSVV, so-called because the inflammation begins with neutrophils, which degranulate and deposit nuclear debris (leukocytoclasis) in the vessel wall.
Hypersensitivity vasculitis: This term was originally used to refer to CSVV but is no longer often used because the etiology of CSVV is rarely hypersensitivity. Hypersensitivity vasculitis, on the other hand, is occasionally used to describe CSVV produced by a recognized medication or illness.

Is Cutaneous Vasculitis that Common?

CSVV can strike at any age, however it most commonly affects those over the age of 16. The condition affects both men and women equally, while some research suggest that men are somewhat more likely to catch it. Caucasians are the most commonly affected.

Every year, an estimated 38 to 55 million persons are diagnosed with CSVV. CSVV in adults was reported to be 45 cases per million in a 2014 population-based investigation in Minnesota. CSVV has a documented incidence of 10 to 30 cases per million individuals per year in Spain, according to research.

Cutaneous Vasculitis Causes

The specific etiology of cutaneous necrotizing vasculitis is unknown. According to one review, 45-55 % are of unknown origin (idiopathic), 15-20 percent are a response to infection, another 15-20 percent are the result of connective tissue diseases, 10-15 percent are reactions to drugs and/or medications, and about 5% are responses to the presence of cancer cells.

Some lesions can be produced by an allergic reaction or hypersensitivity to medications like sulfa or penicillin, as well as other drugs, toxins, and inhaled environmental irritants. Skin symptoms can also be caused by a fungal infection, parasites, or viral infections, and in certain cases, an autoimmune condition.

Autoimmune disorders occur when the body's natural defenses (e.g., antibodies) against "foreign" or invading organisms begin to attack healthy tissue for unclear reasons.

Risk Factors for Cutaneous Vasculitis

Vasculitis can happen to anyone. Factors that may increase the risk of certain disorders include:

Age. Giant cell arteritis is uncommon before the age of 50, but Kawasaki illness is most frequent in children under the age of five.
A family history. Behcet's illness, granulomatosis with polyangiitis, and Kawasaki disease are all conditions that can run in families.
Choices of lifestyle. Cocaine use increases your chances of acquiring vasculitis. Tobacco use, particularly among men under the age of 45, can raise your risk of Buerger's disease.
Medications. Medications such as hydralazine, allopurinol, minocycline, and propylthiouracil may sometimes cause vasculitis.
Infections. Having hepatitis B or C increases your chances of developing vasculitis.
Immune malfunction. People who have immune system problems in which their immune systems wrongly assault their own bodies may be more susceptible to vasculitis. Lupus, rheumatoid arthritis, and scleroderma are a few examples.
Sex. Giant cell arteritis is much more common in women, while Buerger's disease is more common in men.

Signs & Symptoms

Cutaneous necrotizing vasculitis is a frequent condition characterized by inflammation of blood vessel walls and skin lesions. These skin lesions might be flat and red (macules), nodules, or deeper hemorrhages beneath the skin (purpura). They can appear anywhere on the body, however they are most commonly found on the back, hands, buttocks, inside areas of the forearm, and lower extremities.

These skin problems might occur only occasionally or on a regular basis. They normally last a few weeks and might leave darker patches and scars. Wheel-like lesions that cause intense itching (urticaria) or ring-shaped lesions and ulcers may occur in some situations. In extreme situations, blister-like lesions (vesicles, bullae) may form. Fever, widespread discomfort (malaise), and muscle or joint pain are also possible.

Related Disorders

The following diseases can have similarities to cutaneous necrotizing vasculitis:

Polyarteritis nodosa:

It is a kind of systemic necrotizing vasculitis that includes the original classic polyarteritis nodosa, allergic granulomatosis, and diseases with features of both (an overlap syndrome).

Wegener's granulomatosis:

It is a rare collagen vascular disorder that starts with a localized inflammation of the upper and lower respiratory tract mucosa and proceeds to a widespread inflammation of the blood vessels (vasculitis) and kidneys (glomulonephritis).

Churg-Strauss syndrome:

It is an uncommon condition that can affect several organ systems, most notably the lungs. The condition is distinguished by the creation and buildup of a disproportionately high number of antibodies, abnormal clustering of specific white blood cells (eosinophilia), inflammation of blood vessels (vasculitis), and the development of inflammatory nodular lesions (granulomatosis).

Many Churg-Strauss syndrome patients have a history of allergies. Furthermore, the development of generalized symptoms and observations in Churg-Strauss syndrome is frequently preceded by asthma and other related lung (pulmonary) problems. Asthma is a chronic respiratory condition characterized by inflammation and constriction of the airways of the lungs, resulting in difficulty breathing (dyspnea), coughing, the production of a high-pitched whistling sound when breathing (wheezing), and/or other symptoms and findings.

Henoch-Shonlein purpura:

It is a series of disorders distinguished by discolored purple or brownish-red skin. These defects might be huge or minor. Internal bleeding can occur in a variety of locations across the body. This blood vessel issue can cause problems with the skin, joints, gastrointestinal system, kidneys, and, in rare cases, the central nervous system. Little is known about the etiology of this type of purpura, which may be an allergic response that affects children more than adults.

Behcet's syndrome:

It is a rare chronic relapsing inflammatory condition marked by ocular inflammation and mouth and genital ulcers. The reason is unknown. Areas of abnormal skin changes (ulcers or lesions) of the mouth and genitals that tend to recur spontaneously are symptoms. Other bodily systems, such as the joints, blood vessels, central nervous system, and/or digestive tract, may also be affected.

Kawasaki syndrome:

It is also known as mucocutaneous lymph node syndrome, is a juvenile inflammatory condition characterized by fever, skin rash, enlarged lymph nodes (lymphadenopathy), vascular inflammation (polyarteritis), and blood vessel inflammation (vasculitis).

Inflammatory alterations in blood arteries generate damaging lesions, which can lead to issues with the liver, gallbladder, and, most importantly, the heart. The most common cause of acquired cardiac disease in children is Kawasaki syndrome. The specific etiology of Kawasaki syndrome is uncertain, however it may be linked to two previously undiscovered staphylococcus and streptococcus bacterium strains.

Hypocomplementemic urticarial vasculitis:

It is an uncommon disorder marked by repeated bouts of vasculitis and (urticaria) accompanied by fever, arthritis, and abdominal pain. Other symptoms include swelling (angioedema), ocular inflammation, and/or inflammation and degeneration of the capillary clusters (renal glomeruli) that filter the blood as it passes through the kidneys (glomerulonephritis). A chronic obstructive lung condition may occur in some circumstances. It is uncertain what causes hypocomplementemic urticarial vasculitis.

Primary angiitis of the central nervous system (CNS):

It also known as (isolated) vasculitis of the CNS, is an exceedingly uncommon ailment defined by vasculitis of the CNS as a primary condition (i.e., not secondary to another disorder). The physical findings and symptoms of this condition are determined by the individual blood vessels of the central nervous system that are damaged.

There are other conditions that may be characterized by inflammation of certain blood arteries (vasculitis). Giant cell arteritis, polymyalgia rheumatica, lupus, essential mixed cryoglobulinemia, cutaneous necrotizing vasculitis, Cogan's syndrome type I, Takayasu's arteritis, and rheumatoid arthritis are among these illnesses.

Cutaneous Vasculitis Diagnosis

Your doctor will most likely begin by obtaining your medical history and performing a physical examination. He or she may request that you undertake one or more diagnostic tests and procedures in order to rule out or diagnose vasculitis. The following tests and procedures may be performed:

Blood tests. These tests seek for markers of inflammation, such as elevated C-reactive protein levels. A total blood cell count can determine if you have an adequate supply of red blood cells. Blood testing for antibodies, such as the anti-neutrophil cytoplasmic antibody (ANCA) test, can aid in the diagnosis of vasculitis.
Imaging studies. Non-invasive imaging methods can aid in determining which blood arteries and organs are involved. They can also assist your doctor in determining how well you are responding to treatment. X-rays, ultrasound, computed tomography (CT), magnetic resonance imaging (MRI), and positron emission tomography (PET) are among imaging procedures used to diagnose vasculitis.
X-rays of your blood vessels (angiography). A flexible catheter, similar to a thin straw, is placed into a major artery or vein during this surgery. The catheter is then injected with a particular dye, and X-rays are obtained while the dye fills the artery or vein. On the resultant X-rays, the outlines of your blood vessels are evident.
Biopsy. A small sample of tissue from the affected area of your body is removed during this surgical operation. Your doctor will then look for evidence of vasculitis in this tissue.

How is Vasculitis Treated?

Glucocorticoids:

Glucocorticoids (prednisone, prednisolone, and others), sometimes known as "steroids," play an essential role in the treatment of most types of vasculitis. The dose and duration of treatment are determined by the severity of the condition and the length of time the patient has had it. These medications assist to decrease inflammation, but they can have long-term negative effects.

Other drugs:

Immunosuppressive drugs are occasionally prescribed by doctors because their long-term side effects are less severe than those of glucocorticoids. This is known as "steroid-sparing" therapy. Cyclophosphamide is one of the most potent of these medications, and doctors may use it when severe illness threatens vital organs.

Patients with less severe vasculitis may be given methotrexate, azathioprine, or other immune suppressing medications. These medications are frequently prescribed to treat various rheumatic disorders, although they are also beneficial for vasculitis.

Newer biologic medications meant to treat other autoimmune and inflammatory disorders may potentially be beneficial in the treatment of vasculitis. One of these medications, rituximab (Rituxan), was discovered to effectively treat severe instances of specific types of vasculitis by researchers. Granulomatosis with polyangiitis, microscopic polyangiitis, and cryoglobulinemic vasculitis are examples.

Tocilizumab (Actemra) for giant cell arteritis and mepolizumab (Nucala) for eosinophilic granulomatosis with polyangiitis are two more biologic medicines licensed to treat certain kinds of vasculitis. The FDA has approved avacopan (Tavneos) as a supplement to standard therapy for ANCA-associated vasculitis. Some of the most severe instances of these disorders may need plasma exchange ("plasmapheresis") or intravenous immunoglobulin (commonly referred to as "IVIG").

Surgery:

Surgery is occasionally required for serious vasculitis damage. This might include vascular bypass grafting (a surgery to redirect blood flow around a blockage in a blood vessel). Other possible surgeries, depending on the extent of the injury, include sinus surgery or a kidney transplant.

Can Cutaneous Vasculitis be Cured?

The prognosis is favourable, with 90 percent of patients recovering completely (providing only the skin is involved). After the initial episode, the rash may reoccur at intervals for some time. Even after effective treatment, vasculitis-affected skin might become darker pigmented (brown or black) than unaffected skin. It normally takes months for this discoloration to go back to normal.

How to Cope with Your Condition?

One of the most difficult aspects of living with vasculitis may be coping with drug side effects. The following ideas may be useful:

Understand your situation. Find out everything you can about vasculitis and how to treat it. Know the potential adverse effects of the medications you use, and notify your doctor of any changes in your health.
Stick to your treatment strategy. Your treatment plan may include seeing your doctor on a regular basis, getting more tests, and having your blood pressure checked.
Choose a balanced meal. Eating well might help reduce harmful side effects of your drugs, such as bone fragility, high blood pressure, and diabetes. Select a diet rich in fresh fruits and vegetables, whole grains, low-fat dairy products, and lean meats and fish. If you're taking a corticosteroid, talk to your doctor about whether you should take a vitamin D or calcium supplement.
Obtain regular immunizations. Keeping up with vaccines, such as those for the flu and pneumonia, might help avoid complications from your drugs, such as infection. Discuss immunizations with your doctor.
Workout most days of the week. Regular aerobic activity, such as walking, can help prevent bone loss, high blood pressure, and diabetes, all of which can be caused by corticosteroid use. It is also good for your heart and lungs. Furthermore, many people feel that exercise enhances their mood and overall well-being. If you're not used to working out, begin softly and gradually increase your intensity. Your doctor can assist you in developing an exercise routine that is appropriate for you.
Maintain a strong support system. Family and friends can assist you in dealing with this disease. If you believe it might be beneficial to communicate with other individuals who have vasculitis, speak with a member of your health care team about joining a support group.

How to Prepare Yourself For Your Doctor's Appointment?

Because appointments might be brief and there is frequently a lot to talk, it is best to come prepared. Try to:

Be aware of any appointment requirements. When you book the appointment, ask whether there is anything you need to do ahead of time, such as restrict your food.
Send details about prior tests. If you've been sent to a larger medical facility, ask your primary care physician to provide your prior imaging and biopsy findings ahead of time.
List any symptoms you're having, even if they don't appear to be relevant to the reason you made the consultation.
List any medications, vitamins, and supplements you're taking, as well as any other recent health concerns or substantial pressures you've experienced.
Think about bringing a family member or a friend to the appointment. Someone who is with you can help you recall what the doctor says.

Conclusion

Vasculitis is an inflammation of blood vessels that can affect any area of the body. It can affect arteries, veins, or both. When vasculitis affects tiny or medium-sized blood vessels in the skin, it is referred to as cutaneous vasculitis. Occasionally, cutaneous vasculitis might be a marker of inflammation in other organs (a systemic vasculitis), and more testing may be necessary for a complete diagnosis.

Vasculitis may affect everyone; however particular forms are more prevalent in specific age groups. You may improve without therapy depending on your kind. Most varieties necessitate the use of drugs to manage inflammation and avoid flare-ups.

Treatment focuses on reducing inflammation and addressing any underlying problems that may be causing the vasculitis.

The most frequent type of medicine used to reduce the inflammation associated with vasculitis is a corticosteroid, such as prednisone.