Cutaneous Lupus Erythematosus Information for Patients

What is cutaneous lupus erythematosus?

Cutaneous lupus erythematous (CLE or cutaneous lupus) is the skin form of lupus, an autoimmune disease.  Cutaneous lupus can occur along with lupus in other parts of the body (like the kidneys or lungs, which is called systemic lupus erythematosus, or SLE), and sometimes the rash of cutaneous lupus occurs by itself.

Autoimmune diseases are a type of problem where the immune system (your white blood cells) start attacking your own body parts instead of fighting off bacteria, viruses and other infections.  When the immune system attacks your skin there is inflammation and the rash of cutaneous lupus.

There are many different types of cutaneous lupus.  Dermatologists can usually tell apart the different types by how the rash looks.  Sometimes a biopsy of the skin is needed to be sure a rash is cutaneous lupus and to know exactly which type of cutaneous lupus it is.  The rash of cutaneous lupus tends to be most pronounced on sun-exposed skin, like the face and cheeks.

A very common form of cutaneous lupus is discoid lupus.  The name discoid lupus comes from the rash being shaped like a “disc” on your skin.  Discoid lupus presents with circular, disc-like, scaly, itchy spots, usually on the head and neck.  The scalp and the ears are also common locations.  These spots often leave discoloration (both lighter and darker than the surrounding skin) that can be permanent.  When discoid lesions affect the scalp it can cause permanent hair loss in the affected areas, so early and aggressive treatment is recommended.

Another common form of lupus is acute cutaneous lupus, which presents with a butterfly-shaped rash on the central cheeks and nose.  This type of cutaneous lupus is commonly seen along with signs of lupus in other parts of the body.

The third major subtype of lupus is subacute cutaneous lupus, or SCLE.  This type of lupus usually presents as red scaling rings on the chest, upper outer arms and upper back.  Subacute cutaneous lupus is sometimes caused by a medication reaction.

 I’ve been diagnosed with cutaneous lupus.  Do I have systemic lupus erythematosus?

Sometimes the type of rash (like the acute butterfly rash we just discussed) can give us an idea of how likely you are to also have systemic lupus.  It is important for your doctor to ask you questions about how you are feeling and do some tests (like bloodwork and urine tests) to better answer this question

The bottom line is maybe yes and maybe no.  Although most people who have systemic lupus will have a rash of cutaneous lupus at one point or another, many people with cutaneous lupus will never have systemic lupus.  Fortunately for many people with cutaneous lupus, even if they have joint aches and fatigue, they will never develop lupus in other organs like their kidneys, lungs, brain or heart.  However, people with cutaneous lupus should be screened by their doctor on a regular basis for any signs or symptoms or systemic lupus, because there is a small risk of progressing onto systemic lupus.

 What caused my cutaneous lupus?

The cause of cutaneous lupus (and systemic lupus for that matter!) is unknown at this time.  There are some rare forms of cutaneous lupus that run in families, so there is probably a genetic link.  Also women are more commonly affected than men, so hormones may be involved.  Cutaneous lupus (and systemic lupus) flares after sun exposure, suggesting that ultraviolet light may be a disease trigger.  Sometimes medications, including over the counter acid reflux medicines have been associated with a form cutaneous lupus.

What can I expect?

There is no cure for cutaneous lupus yet.  However in the vast majority of patients this chronic disease can be controlled with medicine and lifestyle changes.  Sun protection is a really important part of treatment, so you can expect to start being careful about sun exposure.   Many patients respond to topical therapies, like creams and ointments applied directly to the rash once or twice daily.   Steroid injections directly into the rash can also be helpful.  These injections are given at the doctor’s office every few weeks.  Other patients will go on to take a medication by mouth.  A commonly used medication is hydroxychloroquine (Plaquenil).  It was first used as an antimalarial medicine and no one knows exactly how it works but it seems to suppress the autoimmune attack that is occurring.  Sometimes, when these prior treatments aren’t effective,  medications that suppress the immune system such as prednisone, methotrexate, azathioprine (Imuran) and mycofenolate mofetil (Cellcept) may also be needed.

You should expect that you will see your physician regularly and have periodic screening (in the form of bloodwork, urine tests and questions about how you are feeling).

Because sun avoidance and sunscreen are effective treatments for lupus, patients with lupus are at risk of becoming vitamin D deficient.  Your doctor may test your vitamin D levels during the winter months and recommend supplementation if your levels are low.

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

 Sun protection is a very important treatment for almost every type of lupus, cutaneous and systemic. For this reason, you should avoid sun exposure.  You can do this by limiting your outdoor activities to early morning or late afternoon (prior to 10am and after 3pm), and wearing sun-protective clothing.  You should wear a sunscreen on exposed skin daily and reapply every two hours as instructed on the sunscreen bottle.  This sunscreen should have both UVB protection (reported as SPF) and UVA protection (called broad spectrum protection).  Daily sunscreen is important because UVA rays can pass through clouds and windows (including most car windows).

Studies indicate that people who smoke have cutaneous lupus that is harder to treat, so stopping smoking can also help improve your skin and help you respond better to your medications.

Is my family at risk?

Cutaneous lupus is not contagious; however, if you have cutaneous lupus your family may have a slightly increased risk of having an autoimmune disease.

What are other resources?

JAMA Dermatology Patient Page