What is leukocytoclastic vasculitis?

Leukocytoclastic vasculitis (LCV) refers to small blood vessel inflammation. It’s also known as hypersensitivity vasculitis and hypersensitivity angiitis.

The word “leukocytoclastic” comes from leukocytoclasia, a process where neutrophils (immune cells) break down and release debris. “Vasculitis” means inflammation of the blood vessels.

When people use the term leukocytoclasic vasculitis, they’re usually talking about small blood vessel inflammation in the skin due to infiltrating, dying neutrophils.

However, the term is misleading for the following reasons:

  • Leukocytoclasia occurs when neutrophils are involved in any type of inflammation — not just vasculitis.
  • Similarly, small vessel vasculitis doesn’t always involve neutrophils. It may include other immune cells like lymphocytes and granulomas.
  • The condition can affect the small blood vessels of any organ. It’s not specific to skin.

Cutaneous leukocytoclastic vasculitis is thought to be a more accurate name. This term, along with acute leukoycytoclastic vasculitis, is often used interchangeably with LCV.

Read on to learn about the symptoms, causes, and treatment of leukocytoclastic vasculitis.

Leukocytoclastic vasculitis causes

LCV has many possible causes. Yet, almost half of all cases are idiopathic, which means the underlying cause is unknown.

In general, it’s thought that immune system issues are involved. Potential LCV causes include:

Allergic reaction

In most cases with a known cause, LCV is caused by an allergic reaction to a drug. Usually, the condition develops 1 to 3 weeks after starting the medication.

LCV has been associated with many drugs, including:

  • beta-lactams
  • erythromycin
  • clindamycin
  • vancomycin
  • sulfonamides
  • furosemide
  • allopurinol
  • nonsteroidal anti-inflammatory drugs (NSAIDs)
  • amiodarone
  • beta-blockers
  • TNF-alpha inhibitors
  • selective serotonin reuptake inhibitors (SSRI)
  • metformin
  • warfarin
  • valproic acid

Sometimes, LCV might be caused by an allergy to a food or food additive.

Infection

Infections are another frequent cause of LCV. Bacterial, viral, and parasitic infections are all possible triggers.

Commonly, it’s due to a streptococcal upper respiratory tract infection. Other causes include:

  • Mycobacterium
  • hepatitis B
  • hepatitis C
  • Staphylococcus aureus
  • Chlamydiatrachomatis
  • Neisseria gonorrhoeae
  • HIV

Autoimmune disorders

Various autoimmune diseases have been associated with LCV, which supports the theory that LCV is related to a problem with the immune system.

Autoimmune disorders connected to LCV include:

  • rheumatoid arthritis
  • lupus erythematosus
  • Sjögren syndrome
  • Henoch-Schönlein purpura

Malignancy

A malignancy is characterized by abnormal cell growth. The cells divide uncontrollably and invade surrounding tissues.

Less commonly, LCV may be linked to malignancies such as:

  • solid tumors
  • lymphoma
  • leukemia
  • myeloproliferative disorders
  • myelodysplastic disorders

Inflammatory bowel disease

LCV may be related to inflammatory bowel disease (IBD), including Crohn’s disease or ulcerative colitis.

In some cases, LCV is caused by drugs that treat IBD. The condition also typically develops years after an IBD diagnosis.

Leukocytoclastic vasculitis symptoms

The most notable symptoms of LCV involve the skin. Usually, this includes a rash characterized by:

  • palpable purpura (raised purple-red spots)
  • pain and burning
  • itchiness
  • bullae (fluid-filled sacs)
  • pustules
  • nodules
  • crusted ulcers
  • livedo reticularis (mottled skin)

Typically, the rash forms on the lower legs. Up to one-third of individuals also develop a rash on the trunk and upper extremities.

The following LCV symptoms are systemic, or more generalized:

  • low-grade fever
  • unexplained weight loss
  • muscle aches
  • joint pain
  • bloody urine or stool
  • abdominal pain
  • vomiting
  • coughing
  • weakness

These systemic symptoms affect about 30 percent of people with LCV. Rarely, LCV also causes kidney inflammation.

Diagnosing the condition

To determine what’s causing your symptoms, a healthcare provider can use several tests, which may include:

  • Physical exam of your skin. Your healthcare provider will check for pain, swelling, and inflammation.
  • Medical history. This helps your healthcare provider figure out if something specific triggered your symptoms.
  • Blood tests. Blood tests can show signs of underlying conditions. The tests may include a complete blood count, basic metabolic panel, and liver and kidney function.
  • Urinalysis. A sample of your urine might be checked for signs of disease.
  • Punch biopsy. A healthcare provider takes a small skin sample with a circular tool. The sample, which includes deeper skin layers, is examined at a lab.

While a healthcare provider can diagnose LCV through a physical examination, a punch biopsy is often used to confirm the diagnosis.

Leukocytoclastic vasculitis treatment

Treatment begins with removing or treating the underlying cause of LCV. For example, if you developed LCV due to a drug, your healthcare provider will likely have you stop taking it.

It’s important to remember to speak to your healthcare provider first before stopping any prescribed medications.

A mild case of LCV can be treated with home remedies, including:

  • ice packs
  • elevation
  • antihistamines
  • compression stockings
  • rest

However, if your LCV is chronic or severe, you’ll need additional treatments, which may involve:

NSAIDs

NSAIDs can help manage skin and joint pain. They’re available over-the-counter (OTC), so you don’t need a prescription.

Colchicine

Your healthcare provider may prescribe colchicine, which is made from the plant Colchicum autumnale. This oral drug works to manage neutrophils in the immune system.

While colchicine may help skin and joint symptoms, it doesn’t work for everyone. You might need to take it with other medical treatments.

Dapsone

Dapsone is an anti-inflammatory drug used to treat chronic LCV. It helps to reduce inflammation due to neutrophils.

Depending on your symptoms, your healthcare provider might prescribe dapsone along with:

  • colchicine
  • steroids
  • antihistamines

Prescription steroids

Like NSAIDs, oral steroids are used to manage skin rashes and joint pain. Most people respond to a short course of steroids, such as prednisone or methylprednisolone.

If your internal organs are affected, or if you have severe skin lesions, your healthcare provider might recommend intravenous corticosteroids.

When to see a doctor

Leukocytoclastic vasculitis can range from mild to severe. Therefore, it’s recommended that you see your healthcare provider once you notice any symptoms of LCV.

Seek medical attention if you have:

  • painful, burning rashes
  • raised purple-red spots
  • fever
  • unexplained weight loss
  • trouble breathing
  • weakness
  • bloody urine or stool
  • vomiting
  • persisting joint or muscle pain

Takeaway

If you’re diagnosed with leukocytoclastic vasculitis, it means the small blood vessels in your skin are inflamed.

It may be associated with:

  • medication
  • an autoimmune disease
  • an infection

However, usually, there isn’t a known cause.

Your healthcare provider will develop a treatment plan to manage any underlying conditions. If you have skin and joint pain, they can also prescribe medication to help you feel better.