Kelly Cordoro, MD: Diagnosing and Treating Pediatric Scleroderma

Every year in June, patients and providers across the United States observe National Scleroderma Awareness Month with the intention of bringing awareness to a disease that is estimated to affect 500,000 people in the US alone.

Similarly, those affected by the disease also celebrate World Scleroderma Day on June 29. The holiday, which was first recognized in 2009, commemorates the life and work of Paul Klee, a Swiss abstract artist who struggled with systemic scleroderma, which over time influenced his artistic style.

Much like Klee’s paintings, the understanding of scleroderma has, in some ways, become mysterious, even as new data emerged.

In an interview with HCPLive, Kelly Cordoro, MD, Society for Pediatric Dermatology Fellowship Directors Chair, University of California, San Francisco, spoke of pediatric cutaneous scleroderma, how the disease typically manifests in children, and the limited but promising therapeutic research that has been amassed in recent years.

“It's really different than systemic scleroderma because different parts of the body are involved, and there's a difference (regarding) the prevalence,” Cordoro said. “This is also a very rare condition; the main onset of pediatric cutaneous scleroderma is somewhere between 7 and 8 years of age. So, these are little children that we see, but often there can be a delay in diagnosis of several years.”

Cordoro also noted a gender predilection related pediatric cutaneous scleroderma, with research indicated that more female patients are affected by the disease than male patients. Notably the prevalence of linear scleroderma- which typically manifests in linear swaths across the arms, legs, and midline of the body and face- has also been shown to be more prevalent in younger patients.

Despite uncertainty regarding a genetic predilection of this condition or the environmental factors associated with it, the methods in classifying pediatric scleroderma have significantly improved in recent years.

Naturally, these improvements have led to an increase in early diagnosis as well as continual progress regarding therapeutic maintenance of the disease. While there is currently no cure for scleroderma, topical therapies and calcineurin inhibitors have been effective in management of the disease, and Janus kinase inhibitors (JAK) have shown great promise in various inflammatory conditions.

“Scoring systems to identify the impact of this condition so that we can then follow treatment and response to treatment have really come a very long way,” Cordoro noted. “And there's a lot of researchers and clinicians like myself that are starting to understand and recognize and work towards understanding the etiology and the pathophysiology (of scleroderma) …the earlier we can get successful treatment on board, the more likely the patient is going to have not only a better aesthetic outcome, but also a functional outcome.”

In addition to the physical implications of scleroderma, Cordoro cited an increased focus on the psychological complications as well.

Previous research has detailed the association between scleroderma and depression and other mood disorders, conditions that appear to be more severe in younger patients.

Fortunately, the scleroderma community has continued to address the mental and emotional complications associated with the disease through a myriad organizations, which Cordoro considered to be particularly important in the fight against the disease.

“Patient advocacy groups are one of the most important ways we can spread the word, because knowledge of this condition leads to recognition of this condition,” she said. “I'm involved with the Pediatric Dermatology Research Alliance, or PeDRA, that's very closely associated with the Childhood Arthritis and Rheumatism Research Alliance. Through these groups, we engage with families, caregivers of children that are impacted adults who were diagnosed as children in order to spread the word out about this condition. But the truth is, is that many children continue to be undiagnosed or mismanaged largely because of a lack of recognition of how this disease can be very progressive.”

To hear more on the future of scleroderma care from Dr. Cordoro, watch the full video interview above.