Living with Lupus: Fact #22 – Not so beautiful butterfly rash!

Living with Lupus: The malar, or “butterfly” rash on the face is present in about one-third of those with systemic lupus. This flat, reddish rash across the bridge of the nose and cheeks, is often the only visible symptom of this form of lupus.  Before I was treated for lupus, a malar rash showed up on my face many times.  As a teenager, my mother was intrigued by the deep purple splotches I had over my cheekbones.

Faces of malar rash

Malar rash is one of the eleven diagnostic criteria for lupus.  After my treatment with Plaquenil, the standard medication for all lupus patients, most of the vivid pigmentation of malar rash quieted down.  Now, malar rash shows up after getting too much sun, or during a flare. To prevent over exposure to sun, I wear a large hat and just try to stay out the heat of the day.

Is it malar rash or rosacea?

The Lupus Foundation of America (LFA) provides this important discussion about malar rash, and compares it to rosacea:

“The butterfly blush or rash is a faint or prominent red rash over the malar area and the bridge of the nose. The rash does not go into the sides of the nostrils or down the fold between the nose and the outer part of the lips. These areas are always spared and look white in contrast to the red rash of the cheeks and bridge of the nose. Sometimes the rash is flat, and sometimes it is elevated. It may be in the form of red blotches or may be completely red over the area. The rash may be itchy, especially if it looks more like a rash than a blush. This rash is typical of SLE but is present in only about 30 percent of patients.

The butterfly rash is often confused by patients and by physicians with a similar red rash which also is over the cheeks. This rash is called acne rosacea. It does involve the areas of the outside of the nostrils and does involve the folds between the nose and the outer part of the lips. In addition, pimples may be seen on top of the red rash in acne rosacea.”

Malar rash or rosacea?

Treating malar rash is best accomplished by treating the lupus, and usually Plaquenil is prescribed.  Patients are encouraged to stay out of the sun to avoid excessive UV exposure, which triggers the rash.  Here are some tips the LFA recommends for reducing sun exposure:

1. The first rule is to stay out of the sun, especially during the middle of the day.
2. The second rule is to wear a good protective sunscreen of SPF 30 or higher. Use the sunscreen on all exposed skin areas, including the hands.
3. The third rule is to wear a hat with a broad brim.
4. The fourth rule is to wear long sleeves.

Not make up, it is malar!

Lupus malar rash and an unforgettable social reaction!

Once, I had very uncomfortable and unforgettable moment at work because of my malar rash.  A woman who really didn’t like me or our managing attorney very much began belittling me in front of other co-workers.  I was bewildered as this woman began broadcasting her catty critique, “didn’t you get a little too carried away with your rouge this morning?” assuming the unbalanced redness on my cheeks was because I had used my cosmetics without skill.

I will never forget standing there, with my mouth gaping, wondering what in the world she was talking about! I wasn’t even wearing any cosmetics that day, not even lipstick.  Unbeknownst to me, my malar rash had erupted during my morning bus ride to work from the sun exposure during my three block walk to my office.  I had not looked in mirror since leaving my house, so I was caught completely off guard by her stinging comment.

Forgive the unkind, unaware, rude people

This woman had an unkind habit of making fun of people to try to lower other people’s opinions of them, I suppose in misguided efforts to try to make herself  look better.  It is sad that some people think that making fun of someone who is different, albeit diverse from themselves, is somehow a social “sport.”  I think that people who act this way should actually be pitied, and even prayed for, but not hated — because of their lack of wisdom, kindness, insight and understanding.  They reveal the true shallowness of their own soul by their rude acts, and they completely miss out on the rich blessings that kindness produces in relationships.

Fortunately for me, a couple of coworkers who were the intended audience for her unkind “put down,” instantly arose to my emotional defense.  Without me saying a single word in retort, a couple of other coworkers immediately engaged the woman and publicly shamed her for her rudeness toward me.  They spoke directly to her and harshly condemned her behavior!  Others standing by in the room joined in a spontaneous chorus of rebuke.  My coworkers who knew about my lupus recognized it as the cause of my overly red cheeks. After confronting my critic, they turned to me with many encouraging comments.  As for me, for once, I was speechless!

Grateful for lupus awareness

In retrospect, I was very thankful most of my coworkers were lupus aware, and cared enough about me to take up my cause and handle this woman’s rudeness through a little well-timed peer pressure.  Later, I overheard a “water cooler” discussion among other members of the office about their disbelief and disapproval of the woman’s social crudeness, judging it grossly inappropriate for the woman’s supposed professional, economic and social class.

This woman’s stack of degrees and professional position did not impress anyone that morning, but lupus malar rash certainly had an unexpected impact on my day.

[NOTE:  This post was inadvertently published unfinished at 7:00 a.m. using the pre-scheduled posting feature, before I had really finished writing it and including the personal story I felt was very important to share.  When I realized that this morning, I went ahead and completed it over coffee this morning, and re-published the updated version at 9:30 a.m.  My apologies for making major changes to a post after its initial release.  LA 5-22-2012]

Coping with Lupus: Fact #16 – Discoid is the most common lupus

Coping with Lupus: Discoid lupus (the most common form of skin lupus) accounts for approximately 10 percent of all cases of lupus and occurs in 20 percent of those with systemic lupus.  My discoid lupus was most pronounced before I began taking Plaquenil, and after this first baseline drug treatment started, my rashes cleared up on my face, eyelids, hairline, scalp, chest, arms and thighs.  Now, occasionally a few discoid rashes between my fingers, on my forehead or cheek, or my hairline.

Cutaneous lupus

Forms of cutaneous lupus

The Lupus Foundation of America describes forms of cutaneous lupus in an article on photo-sensitivity and lupus:

1) Acute cutaneous lupus erythematosus (ACLE):

• This is also known as the “butterfly rash” of lupus and occurs over the cheeks and nose.
• It often comes on after sun exposure, and is associated with lupus flares.
• ACLE usually heals within weeks without scarring.

2) Discoid lupus erythematosus (DLE):

• The term “discoid” refers to the disk-shaped lesions of the rash.
• The rash occurs mainly on sun-exposed sites.
• The lesions develop slowly and heal over several months, and may cause scarring.

3) Subacute lupus erythematosus (SCLE):

• SCLE is highly photosensitive.
• It usually shows up as many red, circular shapes on the chest, back and arms.
• It is often a little scaly, resembling psoriasis.
• This form of lupus is particularly associated with antibodies in the blood to the Ro protein (mentioned above).
• SCLE tends to heal over weeks or months and is usually non-scarring.
• It frequently comes back after more sun exposure.

Both SCLE and DLE may occur on their own without the presence of systemic lupus.  Although systemic lupus occasionally develops in people who first have DLE or SCLE, it tends to be a milder illness than the usual form of SLE.

Topical steroids

Slather on the steroids!

A Lupus Foundation of America research report about a new drug, Efalizumab, in the Treatment of Discoid Lupus Erythematosus discusses the following about discoid lupus.

Discoid lupus is a form of lupus that affects the skin (cutaneous lupus). In most cases the discoid lupus rash appears on the face, neck, or scalp, though it can also show up on other areas of the skin. Severe discoid lupus may result in scarring. The treatments that are used most often for severe discoid lupus are strong immunosuppressants that may have significant side effects, especially when used over long periods of time.

A search on www.webmd.com for drugs used to treat discoid lupus, returned a list that included oral Plaquenil/hydroxychloroquine (brand name/generic,) Thalomid, and Thalidomide, the injectable drugs Kenalog, triamcinolone acetonide and Aristospan Intralesional, and a very long list of 106 different topical steroid medications for application to the lupus discoid skin lesions.

Drugs for Cutaneous Lupus

Thalidomide is a drug that has a strong negative stigma about its known relationship to birth defects in the babies of women who used it during pregnancy for their morning sickness. It has been found to be effective in treating some cases of lupus.

Diagnosis and Treatment of Lupus: Fact #12 – Lupus requires multiple medicines!

Diagnosis and Treatment of Lupus:  Successful treatment of lupus often requires a combination of medications. A new treatment for lupus was approved recently, and nearly two-dozen clinical studies are underway to develop a full arsenal of treatments. My treatment plan and combination of drugs used to control lupus and its various symptoms has changed over the years to respond to the variations in my lupus activity, symptoms and organ involvement.

First, aspirin

Many patients with mild to moderate lupus are taking over-the-counter anti inflammatory drugs as a first defense in controlling the symptoms of lupus arthritis and its pain, stiffness and joint swelling. For years I was treated for arthritis of unknown cause, after repeatedly ruling out rheumatoid arthritis through many negative lab tests for it. My family doctor prescribed 10 aspirin per day, in trying to control my inflammation. This was about five years before my lupus diagnosis, when I was new mom in my mid twenties.

Next, Disalcid

Next, my doctor tried the drug Disalcid, after aspirin was inadequate and began causing bleeding from my mucous membranes.   Next, my doctor had me try many non-steroidal anti-inflammatory drugs (NSAIDs) bit I was unable to tolerate most of them. Naproxen caused sharp abdominal pain and Ibuprofen caused bleeding similar to the effects of aspirin.

Then, Sulindac

Eventually, my family doctor put me on an old seldom prescribed anti-inflammatory drug Sulindac because of my intolerance for all the other NSAIDs we had tried.  This was well-tolerated and seemed to control joint inflammation, and helped keep my knuckle joints from ballooning.  This drug was continued for over twenty years, even after my lupus diagnosis and treatment began with lupus medications, because it was still so well tolerated and effective.  Attempts to withdraw this anti-inflammatory medication over the years always resulted in the return of pronounced joint swelling.

Repeated steroid treatments

During those early pre-diagnosis years, there were recurring bouts of discoid raised skin rashes treated with topical steroid ointments and occasional oral and injected steroids.  The multiple use of steroids for the undiagnosed lupus symptoms became problematic, and the doctor began being concerned heavy recurring use of steroids was not medically responsible.  We were frustrated with no answer for my arthritis symptoms, mouth and nose ulcers, rashes and other symptoms before the diagnosis of lupus was finally reached.

After diagnosis, Plaquenil, Sulindac and Tylenol

First post-diagnosis treatments included Plaquenil, continued Sulindac, prednisone and extended-relief Tylenol medications.  For many years this was adequate with bursts of steroids or injections when symptoms would flare and increase from time to time.  There was a gradual worsening of my lupus over time, and eventually stressful life events helped trigger organ involvement in my central nervous system, peripheral neuropathy, liver and overall increased systemic inflammation, joint pain and broad spread lupus activity.

Eventually, Methotrexate and Imuran

For the next ten years, my treatment plan included adding weekly Methotrexate treatment and daily Imuran doses to control the more severe symptoms, but eventually even this combination of medications were unable to control my worsening liver and central nervous system symptoms.

Rituximab, remission and now Benlysta

I entered a clinical trial of the biologic drug Rituximab, and quickly entered into a five-year near remission of the worst of my lupus symptoms.   All my lupus medications were withdrawn except for baseline Plaquenil, occasional Tylenol and smaller short steroid intervention for occasional mild flares.

Recent stressful life events ended the extended remission, and recently the new biologic drug Benlysta became a mainstay of my lupus treatment.

Benlysta was approved by the FDA in March of 2011, and is the first new drug in over 50 years to be approved by the FDA for treating systemic lupus.  Prior to this, only three other drugs were FDA approved for systemic lupus:  Aspirin, Prednisone and Plaquenil.   I am currently receiving monthly Benlysta infusions and have experienced moderate gradual reduction in lupus activity with this medication.

Many drug combinations for lupus

Many other drug combinations are used to treat different lupus patients, and the drugs that they need change over time as their lupus activity and symptoms change.

To read more about drugs and combinations of therapies used in treating lupus, check out the information at the Lupus Foundation of America on this subject.