Cutaneous Lupus

What is cutaneous lupus?

Cutaneous lupus, or skin lupus, is an autoimmune disease that affects your skin, causing skin rashes. Autoimmune diseases happen when your immune system mistakenly attacks your own body tissues. In cutaneous lupus, your immune system attacks your skin cells, causing chronic inflammation in your skin. This manifests as a recognizable rash, often with related symptoms like itching, irritation or swelling.

You may be familiar with another autoimmune disease called systemic lupus erythematosus (SLE). This is a version of lupus that affects your whole body. It may cause inflammation in your joints, blood vessels and vital organs, as well as your skin. Systemic lupus erythematosus can also cause the characteristic lupus rash. Cutaneous lupus erythematosus (CLE) refers to the skin symptoms of lupus by themselves.

Cutaneous lupus is two to three times more common than systemic lupus. But some people with cutaneous lupus do go on to develop systemic symptoms. Sometimes CLE is the first sign of SLE.

Types of cutaneous lupus

Cutaneous lupus can manifest in different ways. For some people, symptoms appear and disappear suddenly. For others, they go on indefinitely, getting better or worse. Cutaneous lupus can also cause different skin lesions with different characteristics. Many of these only occur with lupus. Healthcare providers classify skin lupus by how often you have symptoms and the types of skin lesions you have.

Types include:

Chronic cutaneous lupus. Chronic CLE causes steady skin symptoms, which may get better or worse but never go away completely. The lesions it causes may leave permanent scars or skin discoloration.

Subtypes include:

• Discoid lupus.
• Lupus profundus (lupus panniculitis).
• Chilblain lupus.
• Lupus timidus (intermittent cutaneous lupus).

Acute cutaneous lupus. Acute CLE is a lifelong condition, but the symptoms occur in an acute manner. That means they come on suddenly and then disappear for a while. It doesn’t tend to leave scars.

Subtypes include:

• Localized ACLE (malar rash).
• Generalized ACLE (maculopapular rash).
• Toxic epidermal necrolysis-like ACLE.

Subacute cutaneous lupus. Subacute CLE occurs for limited periods, but repeatedly. For example, it may return every summer with sunlight exposure. It affects a deeper layer of skin, below the epidermis.

Subtypes include:

• Annular SCLE.
• Papulosquamous SCLE.

What are the symptoms of cutaneous lupus?

Cutaneous lupus causes several specific skin rashes. It can also cause other skin symptoms that aren’t specific to lupus. Sometimes, symptoms extend beyond your skin to your mouth, nostrils and hair.

Common symptoms include:

• Red, scaly areas of skin. They might look like a coin or a ring. They especially occur on sun-exposed areas. Rashes might appear purple or brown on darker skin tones.
• A red rash that spreads across your cheeks and the bridge of your nose, in the shape of a butterfly (malar rash). Rashes might appear purple or brown on darker skin tones.
• Patches of lighter and darker pigment on your skin. These are the remains of lesions that have healed.
• Photosensitivity (skin symptoms worsen with UV light exposure).
• Skin itching or irritation.
• Skin swelling (edema), especially around your eyes.
• Hair loss related to scarring on your scalp.
• Mouth sores or sores inside your nostrils.

Other possible symptoms include:

• Hives.
• Livedo reticularis.
• Raynaud’s syndrome.
• Spider veins.
• Petechiae.
• Red palms.

What causes cutaneous lupus?

Cutaneous lupus erythematosus is an autoimmune disease. This means your immune system causes your symptoms. Your immune system generates antibodies that target certain proteins in your body. This usually works to defend you from disease. But in autoimmune disease, your immune system mistakenly targets your own body tissues. This causes the ongoing inflammation in your skin.

Scientists still don’t understand everything about why this happens. They believe it’s a combination of factors. Certain genetic variations make you more likely to get the disease. Then, certain environmental factors can trigger it. These environmental factors provoke a stress response from your immune system. UV radiation exposure (fluorescent light or sunlight) is the most important trigger of cutaneous lupus.

What are the risk factors for acquiring cutaneous lupus?

Risk factors include:

• Having a female reproductive system, especially during periods of high estrogen.
• Having African American heritage.
• Having certain genes that are common in people with lupus.
• Having another autoimmune disease.
• Severe stress to your immune system (like a serious illness or injury).
• Prolonged use of certain medications.
• Prolonged sun or UV light exposure.
• Smoking.

How do healthcare providers diagnose cutaneous lupus?

If you develop lupus skin symptoms, consult a dermatologist familiar with lupus. They’ll ask about your symptoms and examine your skin. Some lupus rashes are recognizable on sight, but others are less recognizable or resemble other skin conditions. Your provider might need to take a small skin sample to examine under a microscope (skin biopsy). If necessary, they might conduct further tests, including:

• Direct immunofluorescence (lupus band test). This test examines your skin biopsy sample under a fluorescent microscope. They look for a band of tagged antibodies in the sample.
• Blood tests. Your provider may conduct blood tests to screen for systemic lupus. While blood tests alone can’t diagnose lupus, they can detect certain signs, like antibodies related to SLE.

Is there a cure for cutaneous lupus?

There isn’t a cure for CLE, or any autoimmune disease. But medications and lifestyle changes can help reduce your symptoms. For example, limiting your sun exposure can help minimize skin reactions. Treating your skin with medications can help soothe skin reactions when they arise. Your healthcare provider will prescribe treatments based on your symptoms and the type of skin lupus you have.

How do healthcare providers treat cutaneous lupus?

Your provider may recommend:

• Limiting sunlight exposure: Learn to stay inside when the sun is strongest during the day. Wear a wide-brimmed hat, sun-protective clothing and sunscreen when you can’t avoid the sun.
• Vitamin D supplements: Limiting your sunlight exposure can put you at risk of vitamin D deficiency. Your provider will monitor your levels and prescribe supplements if necessary.
• Topical medications: Creams, lotions and ointments can reduce skin inflammation. You may need to apply these daily. Corticosteroid creams and tacrolimus ointment are two examples.
• Injections: Your provider may inject a corticosteroid medication directly into your rash. Steroid injections help reduce inflammation and pain. You may need these injections every few weeks.
• Oral medications: Disease-modifying anti-rheumatic drugs (DMARDs) treat autoinflammatory diseases like lupus. Hydroxychloroquine and chloroquine are most often prescribed for CTE.

What can I expect if I have cutaneous lupus?

Cutaneous lupus is a lifelong condition. But with medical treatment and lifestyle changes, you can learn to manage your symptoms. What your symptoms are like, which treatments work best and how well they work can vary from person to person. Unlike systemic lupus, cutaneous lupus doesn’t cause life-threatening symptoms. It shouldn’t affect your life expectancy. But it can affect your quality of life.

How should I take care of myself?

When you’re living with cutaneous lupus, healthcare providers advise that you:

• Don’t touch your skin lesions. This may irritate your skin more and cause new lesions.
• Use dermatologist-recommended cosmetics to conceal lesions if they bother you.
• Apply sunscreen with an SPF of at least 50 before sun exposure.
• Reduce fluorescent lights in your home and workplace, if possible.
• Consider applying UV-blocking window films to windows with high sun exposure.
• See your healthcare provider regularly to check for signs of systemic lupus developing.