Evaluation and Treatment of Pediatric Localized Scleroderma: Pearls and Updates

Abstract

Localized scleroderma (LS), also known as morphea, is a complex and poorly understood disorder in children. The various clinical subtypes of LS require different approaches to evaluation and treatment. Failure to recognize and appropriately treat the affected skin and underlying deep connective tissue can lead to long-term morbidity. We present a review of the current literature supporting treatment options for pediatric LS followed by a series of cases to highlight the complexity of treatment choices and identify clinical pearls. Increasing evidence has emerged for the utility of systemic immunomodulation in treating localized scleroderma. Multiple consensus-based treatment protocols are now available, though underlying evidence is moderate. There are emerging reports utilizing modern biologic and small molecules, but these remain relatively sparse. Therapeutic regimens for LS should be based upon a detailed physical examination augmented by the results of additional screening evaluations recommended for the subtype of LS. Close collaboration with multiple specialties may be needed to achieve optimal outcomes, including rheumatology, dermatology, physical therapy, and craniofacial plastic surgery. More study is needed to identify the efficacy of newer therapeutic options as well as the role for adjunctive therapies.