Dr. Lauren Graham provides tips for diagnosing and treating rheumatologic skin disease, including cutaneous lupus, dermatomyositis, morphea, and scleroderma.
Lauren Graham, MD, PhD, Associate Professor of Dermatology, University of Alabama at Birmingham, Birmingham, Alabama.
“My talk focused on cutaneous lupus, dermatomyositis, morphea, and scleroderma,” said Lauren Graham, MD, PhD, who presented “Everyday Tips for Rheumatologic Skin Disease,” at the 2023 Alabama Dermatology Society (ADS) Summer Symposium in Miramar Beach, Florida.
Dr. Graham first shared tips about the relationship between systemic and cutaneous lupus, including whether people with cutaneous lupus will develop systemic lupus.
“There have been a lot of studies done on this. Interestingly, the studies use different lupus criteria because the systemic lupus criteria have changed over the years. Also, some of the studies use different follow-up times. The data ranges from 0% (it’s not likely to turn into systemic lupus) to 29% of people with skin lupus could develop systemic lupus.”
One group that looked at 25 studies, found patients were more likely to develop systemic lupus if they had a lab abnormality for a positive [antinuclear antibody] ANA and/or blood lab abnormalities, said Dr. Graham.1
“Interestingly, the authors also found that patients with cutaneous lupus have milder systemic lupus, on average. They’re less likely to have brain or kidney involvement. That’s something that we, as dermatologists, could reassure our patients about.”
When it came to pediatric systemic and cutaneous lupus, Dr. Graham said a paper in which authors reviewed 8 studies found that from 0% to 26% of those with cutaneous lupus developed systemic disease.
“The risk factors for developing systemic lupus in children were if they were older when they developed skin lupus, if they had the positive autoantibodies, and one study showed the greatest risk for developing systemic disease is pretty early on after they develop skin lupus.”
Drug-Induced Cutaneous Lupus
Medications can cause skin lupus. But pinpointing a drug-induced cause can be tricky given cutaneous lupus onset can vary greatly, said Dr. Graham.
“Depending on the drug, it can be from 2 to 3 weeks after you take a drug such as an antifungal, all the way to 2 to 3 years later with blood pressure medicines.”
Treating Cutaneous Lupus
Smoking cessation, sunscreen, and sun protection, are among the best preventive measures for skin lupus, said Dr. Graham.
“The best sunscreens are the ones with physical blockers or tinted sunscreen, which has iron in it. [The iron] often is not in the active ingredients, so you have to look down in the inactive ingredients for iron.”
In some states, dermatologists can write letters that patients take to their drivers’ license bureaus for permission to get darker tinting on car windows. This can offer added sun protection, according to Dr. Graham.
When using hydroxychloroquine to treat cutaneous lupus, note that the American Academy of Ophthalmology changed its recommendations for use in 2016, said Dr. Graham.
“The recommendation now is for the dose to be under 5 mg/kg of a patient’s real body weight. If you’ve had patients on hydroxychloroquine for a long time you may want to reevaluate their dose. Or you might want to reevaluate if patients have lost weight.”
On the topic of antimalarials, Dr. Graham said that the risk for eye toxicity increases if a patient is on tamoxifen or has renal disease. The risk also increases the longer patients are on the drugs.
“Another medicine we use for skin lupus is methotrexate, [which] we use in dermatology for lots of skin diseases. These are good tips if you’re using methotrexate, in general: Consider switching to subcutaneous injections at a dose greater than 15 mg weekly. It helps with both gastrointestinal side effects and with better bioavailability. Another good tip for patients that don’t want shots or are over 15 mg per week, have them split the dose for better bioavailability. I often have them take half their pills in the morning and half their pills at night on that same day, once a week.”
Dermatomyositis
Dermatomyositis can be difficult to treat and to diagnose. Dermatomyositis often looks like cutaneous lupus on skin biopsy, so dermatologists tend to make the diagnosis clinically, said Dr. Graham.
“How do you tell the difference between dermatomyositis and cutaneous lupus? Hands are a good place to look. Often lupus is a rash in between the joints, whereas the rash from dermatomyositis is on the joints.”
“… nasolabial folds are often spared in lupus and affected in dermatomyositis.”
The scalps of dermatomyositis patients are likely very red, pink, erythematous, or scaly. While skin lupus on the scalp can also scale and itch, a diffuse red scaly scalp should make the dermatologists think about dermatomyositis, she said.
Dermatologists also should note that the severe itch of dermatomyositis and all connective tissue diseases does not respond well to antihistamines, said Dr. Graham.
“Gabapentin and pregabalin can be more helpful. Those are the medicines I go to first if I need a systemic medicine for itch in this patient population.”
With dermatomyositis, consider starting a steroid-sparing agent right away, said Dr. Graham.
“These patients can be difficult to treat and often need long-term treatment and/or more than one systemic treatment. So, try to get them [going] right away with … screening labs. Find out about their medical history, social history—all those things right away—because you are most likely going to need a stronger systemic medicine.”
The good news is there’s lots in the pipeline and ongoing clinical trials for these difficult-to-treat skin diseases like cutaneous lupus and dermatomyositis, said Dr. Graham.
Morphea
Pictures speak 1000 words when it comes to monitoring for change in morphea patients, said Dr. Graham.
“Even if they have limited disease, I strongly believe in taking pictures. I encourage them to [take pictures and compare them to their previous pictures] about once a month. Then we see them in follow up we can compare old and new pictures.”
Dr. Graham said she gets a lot of questions about whether to treat morphea.
“If patients are stable and haven’t had any change for several months to years, I often recommend not to start treatment unless the disease becomes active.”
Phototherapy remains one of the most effective morphea treatments. Of note, studies have shown there may be better improvement with UVA1. But there’s also evidence of improvement with narrowband UVB, which is more accessible to most dermatologists, she said.
According to Dr. Graham: “Make sure you check bloodwork in scleroderma patients. Specifically, you want to check for RNA polymerase III antibodies. This is important because if they are positive for this antibody, you do not want to give them systemic steroids. Studies have shown that can increase their risk of renal crisis.”
Lasers can help to address the cosmetic concerns of scleroderma patients with telangiectasias, said Dr. Graham.
“Studies have shown laser treatment on scleroderma patients can take longer and require more treatments than with healthy patients.”
Dr. Graham also addressed the latest vaccine recommendations.
“The American College of Rheumatology published its new vaccine guidelines in 2022 that any patient who is immunosuppressed and over the age of 18 should get the high-dose or adjuvanted flu vaccine. It’s also strongly recommended to get the pneumococcal vaccine. The varicella vaccine also is approved for over 18 if you’re immunosuppressed. But the guidelines can change, so I would always look at the CDC guidelines, too.”
Reference:
- Curtiss P, Walker AM, Chong BF. A Systematic Review of the Progression of Cutaneous Lupus to Systemic Lupus Erythematosus. Front Immunol. 2022;13:866319. Published 2022 Mar 11. doi:10.3389/fimmu.2022.866319.
Disclosures: Dr. Graham is a principal investigator on clinical trials for Pfizer, Biogen, Regeneron, Argenx. She is a consultant for Clarivate and Argenx.
