From the Inside Out: The Many Faces of Cutaneous Lupus

By Elizabeth Thompson Beckley

Rachelle Pamela sought medical attention for a mouth ulcer when she was 19 years old. It took several biopsies, many incorrect conclusions, various treatments, and another eight years before, at age 27, Pamela received the correct diagnosis of discoid lupus erythematosus.

Discoid lupus is the most common of the skin diseases that fall under the umbrella of cutaneous lupus. “Cutaneous” means “of the skin.” Only about 10 percent of people with discoid lupus go on to develop systemic lupus, but about 80 percent of people with systemic lupus will also have skin problems.

Cutaneous lupus can affect the skin in many ways. Rashes, mucosal ulcers (sores in the mucous membranes of the mouth and nose), bumps, and dimpling can be painful or itchy. In some cases, the effects on the skin may result in permanent discoloration, scarring, and hair loss—aspects of appearance that also can take a toll on the psyche.

The various eruptions of cutaneous lupus usually are referred to as lesions. A lesion is an abnormality in tissue or an organ caused by disease or injury.

Lesion Lesson

There are three types of cutaneous lupus, but not all of them cause scarring. According to David Fiorentino, M.D., Ph.D., assistant professor in the department of dermatology and the department of medicine’s division of immunology and rheumatology at Stanford University School of Medicine in Palo Alto, CA, the main type of scarring is caused by lesions from discoid lupus.

Acute cutaneous lupus, which frequently appears as a rash across the nose and face (often called the “butterfly” rash), doesn’t scar, Fiorentino says.

Subacute cutaneous lupus doesn’t scar, but can leave white areas where there was a rash and the skin’s pigment has been lost (hypopigmentation) or darker areas where the skin’s pigment has become discolored (hyperpigmentation).

“Even though skin problems often are associated with lupus, we can’t always assume the skin rash is related to lupus,” Fiorentino says, “but the type of rash can be a signal of how active or inactive the internal lupus is.”

Further analysis is required to determine an individual’s overall health. Often a biopsy of the affected tissue—done by taking a tissue sample and examining it under a microscope—can give more information.

For Pamela, now age 40 and an office manager for a trucking company in Concord, CA, aggressive treatment has kept her discoid lupus at bay. She now maintains her skin’s health with a manageable drug regimen of antimalarials and a topical steroid.

“I take two pills [chloroquine and quinacrine] in the morning and use a cream [fluocinonide] at night,” she says. They’re paid for by insurance, and Pamela reports experiencing no side effects. “That’s the great part,” she says.

The not-so-great parts are the scarring and discoloration that remain from previous flares, which she describes as “a lot of pits and indented scars” around her lips, chin, ears, and scalp.

Treatment Strategies for Skin

Fortunately, several effective treatments for cutaneous lupus are available. There also are new drugs in the research pipeline, as well as new techniques for dealing with scars and hair loss.

Because so many lupus lesions are photosensitive, meaning the skin is very susceptible to damage and outbreaks triggered by the sun’s ultraviolet light (or, less often, by UV light from artificial indoor lighting), the first line of defense is almost always against those UV rays.

“The cornerstone of therapy, before we talk about any medication, is how to protect yourself from sun,” Fiorentino says.

A red rash on her face, which she thought was a reaction to an antibiotic, sent Ariceli Flores to her physician two and a half years ago. Tests confirmed that she had systemic lupus, and she has since experienced discoid lesions on her arms, ulcers in her mouth, and hair loss.

“Since I have this rash on my face, I’ve been getting used to wearing what we call the goofy hats—the bigger hats with the wider brim—because I know in the long run it will benefit my skin, and that’s been helping,” says Flores, a 26-year-old nutrition educator for a local school district in Salinas, CA. “One of my co-workers, who works with me in the garden, always says, ‘We’ve got to get our goofy garden hats on now.’ You feel funny wearing it, but you get used to it.”

According to Richard D. Sontheimer, M.D., professor and vice chairman in the department of dermatology at the University of Oklahoma Health Sciences Center in Oklahoma City, treatments for cutaneous lupus will vary, depending on the type and extent of skin involvement.

He says that the standard treatment path begins with topical (cream or gel) corticosteroids, especially for mild cases of acute and subacute cutaneous lupus. Other options are topical calcineurin inhibitors.

Injection of a steroid directly into the lesion also can be helpful, Fiorentino says, especially with localized discoid lupus. “We tend to not use a topical treatment for discoid lupus because [the lesions] go too deep in the skin for a cream to get to,” he says. For those with limited disease, the only therapy may involve an injection of cortisone, but two or three monthly injections may be necessary to heal the lesions.

In terms of a systemic drug, antimalarials are the first-line therapy for most people with cutaneous lupus, the experts agree. “In my opinion, they all should be on an antimalarial unless they can’t be because of allergic reactions or other contraindication,” Fiorentino says. “We really like to use them because they are so safe.”

Hydroxychloroquine, chloroquine, and quinacrine are the three antimalarials used in North America (although quinacrine is only available from compounding pharmacies, where the drugs are made by the pharmacist).

However, people taking an antimalarial shouldn’t smoke, Fiorentino notes, because smoking keeps the drug from working as effectively. He adds that there is also a rare chance of toxicity to the eye, so anyone taking an antimalarial should have regular eye exams from an ophthalmologist.

Generally, drugs that treat the underlying lupus will help clear the skin symptoms. So, for people who don’t respond to topical treatments and antimalarials, some second-line medicines—for example, oral steroids or immunosuppressants such as methotrexate and azathioprine—may be successful.

Thalidomide is another important drug option. It has been shown that 80 percent of people who don’t respond to antimalarials will respond to thalidomide, Fiorentino says. Because of the known risk for birth defects with the drug, women in their childbearing years who take thalidomide are cautioned to use two forms of birth control.

One of the newer drugs being used for cutaneous lupus is mycophenolate mofetil (CellCept®). Developed to prevent rejection of organ transplants, CellCept already has shown promise as a treatment for both lupus kidney disease and lupus that involves the nervous system.

“And now we’re finding out that CellCept can be helpful for skin disease as well,” Fiorentino says. “It is becoming one of our go-to therapies after antimalarials fail. Expense is the main issue. It can be hard to get insurance companies to pay for it.”

Rituximab (Rituxan®) is another new agent that dermatologists are turning to for people whose cutaneous lupus has not responded to usual therapies. Rituximab is a monoclonal antibody originally designed to treat lymphoma, and its off-label use for lupus and other autoimmune conditions is relatively new. More than a year ago, Flores was treated with an injection of rituximab. Together with the oral steroid, prednisone, she says the rituximab (which she will take again this year) has helped put her lupus in remission and clear her skin rash and mouth ulcers. Again, though, its off-label use presents expense problems with insurers.

Several new therapies to treat various forms of cutaneous lupus are being studied, Sontheimer notes. You can find out more about these by visiting lupus.org, the Web site of the Lupus Foundation of America, or by searching for “cutaneous lupus” at www.clinicaltrials.gov.

Must Scars Be Forever?

The best chance to prevent scarring and hair loss is to treat even the smallest lesions immediately, especially with discoid lupus. But once a scar has been established, there are some treatment options, depending on the type of scar. For instance, uneven-looking skin would be treated differently than red marks from dilated blood vessels or dark areas from hyperpigmentation.

“All of these can be cosmetically unacceptable to the patient, and you need to find out from them what it is about the mark that they don’t like,” Fiorentino says.

“Once that is established, the disease must be inactive before you think of treating a scar,” he says. “If you injure the skin with laser treatment, for example, it can trigger the lupus and more new lesions.”

“I make sure [my lupus patients] are at least on an antimalarial and have been in remission for at least six months,” Fiorentino says.

To reduce unevenness, dermatologists have been using the resurfacing laser for many years. Dermabrasion, or sanding of the skin, is another possibility. For red marks, other types of lasers target the blood vessels and usually are well tolerated. Bleaching creams and different types of lasers to treat darker areas entail working with a very experienced cosmetic surgeon who should work in close collaboration with the physician treating your lupus.

Fiorentino recommends testing a laser treatment on a small area to make sure the treatment can be tolerated and that it doesn’t trigger lupus symptoms.

For discoid lupus that scars the scalp and causes hair loss, a procedure called a scalp reduction is used to remove the scarred area and bring together the areas that still have hair. Small balloons called tissue expanders are placed under the scalp to stretch the skin so there is enough to close over the bald area. Hair transplants are another option.

For the time being, both Pamela and Flores have opted not to pursue the surgical treatments for their skin scarring, choosing instead to surround themselves with people who see what’s under the surface.

“My family and close friends have been very, very supportive,” Flores says. “When they see me get mopey, they come find me and we’ll have dinner or do something just to get out.”

Flores’ advice for others contending with cutaneous lupus? “Keep your hopes up. Having this skin condition has brought me a lot closer to my family and friends, and I just see the world with a different perspective,” she says.

Pamela advises others confronting cutaneous lupus to actively seek appropriate treatment in the beginning and to search for a doctor who works for them.

“I was treated by one dermatologist who didn’t work with me, and the medicine he had me on wasn’t doing any good, so I gave up,” Pamela says. “Now, I have doctors at Stanford who are treating my skin lupus aggressively, which I need. So my advice is, never give up.”

Reprinted from Spring 2008 of Lupus Now with permission of the Lupus Foundation of America, Inc. Copyright 2008

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