Widespread morphea treatment disparities signal need for guidelines

A survey of pediatric dermatologists and rheumatologists reveals wide variation in how patients with pediatric morphea are treated.

The survey highlights the need for physicians to develop consensus treatment guidelines and for more research to determine optimal treatment, write the authors of an article in Pediatric Dermatology, published in November 2017.

An inflammatory autoimmune condition, morphea, or localized scleroderma, often starts in childhood. There are no treatment guidelines for the mild form of the condition, but there are consensus guidelines for moderate and severe morphea.

Treating morphea early and effectively can help to stop its progression, which can include scarring, atrophy, disfigurement, pain and limited functional ability.

While diagnosing morphea can be straightforward, often based on clinical and histopathological findings, determining disease activity can be more difficult.

“Morphea is a rare condition, but one that dermatologists see most commonly. Despite this, there is a lot of variability in how different dermatologists approach morphea, specifically regarding how to decide if a morphea lesion is active since there are not really any reliable objective measures for that and … how to treat,” says the study’s lead author Megha Tollefson, M.D., a pediatric dermatologist with the Mayo Clinic Minnesota.  

Dr. Tollefson

It’s important to describe today’s most common approaches for evaluating and treating pediatric mor phea, as dermatologists and others move toward developing guidelines that will help those who take care of children with morphea, according to Dr. Tollefson.

Dr. Tollefson and colleagues reported on survey results from 110 Society for Pediatric Dermatology members in August 2014. The 17-question survey asked about pediatric morphea treatment practices and presented case-based scenarios.

Nearly all respondents - 99 percent- agreed that red violaceous rim is a sign of morphea lesion

activity. Seventy-five percent indicated local warmth indicates disease activity, followed by 69 percent agreeing raised borders and 64 percent agreeing dermal thickening suggest a child’s morphea is active.

There was less agreement among respondents about whether sclerotic, “porcelain-like” lesions (41 percent), scaling (43 percent) dyspigmentation (19 percent) and atrophy (18 percent) are indicative of disease activity.

The doctors were most likely to use topical medications to treat plaque morphea. More than 90 percent said they used these therapies as primary or the only treatment. But they weren’t likely to use topical therapies to treat generalized morphea.

Among those responding, 45 percent indicated they used topical therapy for linear morphea associated with the limbs and 37 percent for linear morphea of the head or neck. The authors write that the respondents’ reliance on topical therapies for linear morphea, the most common subtype in children, was striking.

“Given the risk of long-term morbidity from linear morphea, these patients may benefit from the use of systemic immunosuppression,” the authors write.

The go-to treatment for 80 percent of respondents: high-potency topical corticosteroids. Other commonly used topical treatments were calcipotriene (73%), tacrolimus (66%). Only 10 percent indicated using imiquimod, 18 percent pimecrolimus 44 percent calcipotriene plus betamethasone and 45% a mid-potency corticosteroid.

Respondents most commonly used one-daily or twice-daily applications of topical corticosteroids, and twice-daily treatment with calcipotriene and tacrolimus.

In most cases, respondents waited one to three months before deciding topical therapies were ineffective.

The big picture is a lack of standardization and consensus in treating patients, even among pediatric dermatologists, the authors write.

The discordance could be more pronounced in the bigger dermatologist community, according to Dr. Tollefson.

“We have not specifically studied pediatric morphea treatment in the general population of dermatologists, but, yes, one may suspect that there would be even more variation in treating morphea in children amongst all dermatologists,”

Dr. Tollefson says. “Morphea treatment in patients of all ages amongst dermatologists has been studied, and there is a great deal of variation there. In addition, rheumatologists also commonly treat morphea, and there is a great deal of variation between how dermatologists and rheumatologists treat morphea.”

Previous research suggests differences in treatment spans medical specialties. Dermatologists and rheumatologists, for example, differ when it comes to treating pediatric plaque morphea.

Dermatologists prefer topicals and phototherapy, while rheumatologists tend to use systemic medications, according to a UK survey cited in the paper.

More research is needed to define the disease, including active disease means, as well as to identify which treatment may work best in which type and location of morphea, according to Dr. Tollefson.

“One area where we can be better is treating linear morphea in children more aggressively.

We tend to hesitate to put children on systemic medications because we rightfully fear the possible long-term side effects, but due to the potential for long-term morbidity with linear morphea, many patients may benefit from systemic treatment,” she says.

Developing guidelines for treatment is the next big step.

“Morphea is rare, but through the pediatric dermatology research alliance (PeDRA), we have been able to form a group of colleagues who are interested and actively working on furthering research in pediatric morphea. While this group is primarily composed of pediatric dermatologists, we have involved adult dermatologists specializing in morphea, and pediatric rheumatology colleagues who have expertise in morphea in many of the studies done through this group,” she says. “There are efforts underway to develop guidelines, to develop tools with which to better define activity and severity and to develop more objective outcome measures that can be used when evaluating potential treatments. This will allow for more consistency when studying potential treatments, so that we can determine whether or not a specific treatment works, and whether or not one treatment is better than another.”

REFERENCE

Tollefson MM, Chiu YE, Brandling-Bennett HA, Pope E. Discordance of pediatric morphea treatment by pediatric dermatologists. Pediatric Dermatology. Nov. 22, 2017. DOI:10.1111/pde.13281.