Survey highlights need for pediatric vulvar and perianal lichen sclerosus guidelines
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Key takeaways:
- The survey (n = 103) included 46% pediatric/adolescent gynecologists and 41% pediatric dermatologists.
- 86% of respondents felt there was a need for better evidence-based management guidelines.
Researchers find that while clinicians agree on diagnostic and treatment practices for pediatric vulvar and perianal lichen sclerosus, management techniques lack uniformity, according to a study.
“Lichen sclerosus (LS) is a chronic inflammatory disease with bimodal age of diagnosis in prepubertal girls and post-menopausal women,” Christine M. Pennesi, MD, director of the MRKH Care Center at the Cincinnati Children’s Hospital Medical Center and assistant professor in the obstetrics and gynecology department at University of Cincinnati, and colleagues wrote. “The symptoms of LS can mimic other conditions and lead to a delay in accurate diagnosis.”
This delay in diagnosis, especially in pediatric patients who have pediatric vulvar and perianal LS (PVPLS), can create complications including persistent disease into adulthood. To learn more about clinicians’ current diagnostic, treatment and management regimens for PVPLS, the authors conducted a cross-sectional 35-question survey.
The survey was administered through the Pediatric Dermatology Research Alliance and the North American Society for Pediatric and Adolescent Gynecology. Of the 103 responders, 46% were attending-level pediatric/adolescent gynecologists and 41% were pediatric dermatologists.
Diagnosis
Results showed that 85% of respondents were confident in their ability to diagnosis PVPLS with a higher level of diagnostic confidence being correlated to more years in practice (P = .0014).
Nearly all participants (99%) reported using medical history and a physical examination alone to diagnose PVPLS.
Also, 51% of pediatric/adolescent gynecologists and 67% of dermatologists noted vitiligo as the most difficult condition to distinguish from PVPLS. Additionally, those who saw fewer than two PVPLS cases per month stated they had trouble distinguishing child abuse from PVPLS.
Treatment
Seventy-seven percent of respondents reported having a standard protocol for treatment with 93% reporting the use of a super high potency topical steroid for initial treatment, 78% of which recommended twice-daily application.
Most pediatric dermatologists suggested initial treatment span 4 to 6 weeks, whereas pediatric adolescent gynecologists recommended shorter time frames of 2 or 4 weeks (P = .03).
Of all the clinicians, 82% reported routinely tapering the frequency of topical agents and 64% reported prescribing a maintenance regimen for quiescent disease.
Management
Ninety-one percent of respondents regularly counsel patients with PVPLS on the importance of gentle skin care and hygiene during follow-ups. While the disease is active, 44% of respondents stated they preferred a 4-week follow-up interval.
After the disease appears under control, 49% of respondents reported reducing follow-up frequency to 6 months, and 36% reduced frequency to every 3 to 4 months.
Forty-three percent of respondents noted that they recommend lifelong follow-ups. On the other hand, 19% of respondents only conducted follow-ups until disease activity resolved, 16% until puberty and 12% as needed.
While 71% of respondents found the current PVPLS guidelines to be helpful, 86% felt there was a need for additional evidence-based management guidelines.
Conclusion
“The variable use of symptoms and examination findings in formulating treatment recommendations underscores the need for standardized tools to categorize disease activity and assess responses to treatment in PVPLS,” the authors wrote. “Future studies are imperative to assess optimal medication regimens and to standardize care protocols for the treatment of PVPLS.”
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