Acute Cutaneous Lupus

What is acute cutaneous lupus?

Lupus is an autoimmune disease where your body attacks itself. One form of lupus is cutaneous lupus erythematosus (CLE). Cutaneous refers to your skin. CLE is an autoimmune disease where your immune system attacks your skin. People with CLE get sores or lesions on the skin of their face, nose and cheeks. Acute cutaneous lupus (ACLE) means that the rashes appear suddenly (acutely), often in response to sun exposure.

What is lupus?

The most common type of lupus is systemic lupus erythematosus (SLE). In SLE, your body’s immune system attacks other organs in addition to your skin, such as kidneys, heart, lungs and joints. SLE is the most common type of lupus. Almost three out of every four people who have lupus have SLE.

SLE causes widespread inflammation. This inflammation might be acute (sudden) or chronic (long-lasting). People with lupus often have:

• Fatigue.
• Joint pain or swelling.
• Rashes or other skin symptoms.

People may have one type of lupus, such as SLE or CLE. But more often, they have multiple types of lupus.

What are the types of cutaneous lupus erythematosus?

There are three major types of cutaneous lupus. Each type causes a different skin rash.

• Discoid lupus causes coin-shaped, thick, dark/red patches on your cheeks, ears or nose.
• Subacute cutaneous lupus causes ring-shaped lesions on your arms, chest, back or neck.
• Acute cutaneous lupus causes a “butterfly rash” that looks like a sunburn across your nose and cheeks.

Who might get acute cutaneous lupus?

Acute cutaneous lupus is common in people who also have systemic lupus (SLE). Cutaneous lupus is generally most common in women ages 20 to 50.

How common is acute cutaneous lupus?

About 2 in 3 people who have systemic lupus also have some CLE symptoms. Around 1 in 10 people who have lupus have only CLE.

What causes acute cutaneous lupus?

Experts don’t know what causes acute cutaneous lupus. Some people get the butterfly rash as their first sign of SLE before they're diagnosed with SLE.

Lupus and other autoimmune diseases may run in families. You may have a higher risk for developing lupus if you are:

• Female.
• Aged 15 to 44.
• Black, Asian American, Hispanic/Latino or Native American.

What are the symptoms of acute cutaneous lupus?

For most people, acute cutaneous lupus appears as a butterfly-shaped rash across your nose and cheeks. Another name for this rash is a malar rash (rash on the cheeks). This rash typically looks like a sunburn and usually doesn’t hurt but may itch.

People may also get rashes on their arms or legs. These rashes often appear after sun exposure, although they are not a sunburn. ACLE skin rashes don't scar, but after they go away, your skin may look lighter or darker than it was before.

Acute cutaneous lupus may also cause:

• Canker sores (painful sores in your mouth).
• Hives (raised, painful bumps).
• Temporary hair loss.

What are symptom flare-ups?

People with ACLE experience symptom flare-ups. Having flare-ups means you don’t have ACLE symptoms all the time. Instead, you develop symptoms that may come and go, such as after exposure to triggers like stress or too much sunlight.

How is acute cutaneous lupus diagnosed?

A dermatologist (doctor specializing in skin) or a rheumatologist a doctor specializing in arthritis) may diagnose acute cutaneous lupus.

Sometimes your provider may do a skin test called a biopsy to make the diagnosis. During a biopsy, your dermatologist removes a small skin sample. Your provider sends the skin sample to a laboratory for examination under a microscope. By looking at the skin sample under a microscope, healthcare providers can tell if a skin rash is due to acute cutaneous lupus or another condition.

How is acute cutaneous lupus treated?

Depending on how severe your symptoms are, your healthcare provider may recommend medications. These medications don't cure lupus but can reduce symptoms and flare-ups.

Some research suggests that medications are helpful for about 60% of people with cutaneous lupus. Lupus rash treatment might include:

• Topical steroid ointments, such as fluocinolone acetonide (Synalar®) or hydrocortisone butyrate (Locoid®), reduce swelling and inflammation.
• Anti-inflammatory drugs such as dapsone (Aczone®) or low-dose methotrexate (Otrexup™, Rasuvo®) reduce pain and swelling.
• Antimalarial drugs, such as hydroxychloroquine (Plaquenil®), fight fatigue, reduce rashes and decrease joint pain.
• Calcineurin inhibitors, such as tacrolimus (Protopic®, Prograf®) or pimecrolimus (Elidel®), decrease inflammation by suppressing your immune system.

How can I prevent lupus rash flare-ups?

About 40% to 70% of people with lupus have symptom flare-ups after exposure to ultraviolet (UV) light. To minimize flare-ups, people with lupus need to take extra caution around sunlight or artificial light.

To prevent lupus butterfly rash flare-ups and protect yourself from UV exposure:

• Apply a broad-spectrum sunscreen with at least SPF 30 every day.
• Avoid sunlight when the sun is strongest, between 10 a.m. and 4 p.m.
• Don’t use tanning beds.
• Limit your time around indoor fluorescent lights.
• Wear sun-protective clothing, such as wide-brimmed hats and long sleeves.

How can I prevent acute cutaneous lupus?

You can’t prevent lupus. But if you have lupus, you can avoid symptom flare-ups by identifying and avoiding triggers.

In addition to avoiding sun exposure, you may also want to:

• Eat a diet full of nutritious foods, such as fruits, vegetables and whole grains.
• Exercise moderately as often as you can.
• Manage stress with healthy coping tools.
• Sleep at least seven to eight hours per night.
• Take all medications as prescribed.
• If you smoke, stop as it can make the rash worse. Smoking may also make medications that treat the rash less effective.

What is the outlook for people with acute cutaneous lupus?

Acute cutaneous lupus is a lifelong condition. Most people experience symptom relief with treatment. You will need to take extra precaution around UV rays to avoid flare-ups.

What else should I ask my doctor?

You may also want to ask your healthcare provider:

• How can I identify lupus symptom triggers?
• What can I do to treat an ACLE rash?
• Should I take any dietary supplements or change the foods I eat?
• How much can I exercise if I have an autoimmune disorder?

A note from Cleveland Clinic

Acute cutaneous lupus is a type of lupus that affects your skin. People with ACLE often have rashes that look like a sunburn. These rashes usually affect your nose and cheeks. For many people, avoiding sun exposure can help to prevent severe flare-ups. Your healthcare provider may also prescribe medications to reduce the frequency and severity of symptoms. Lupus is a lifelong condition, but treatment can help you manage symptoms.