Morphea is characterized by sclerosis of the skin and, in some cases, underlying tissue. It is generally thought to be an autoimmune disorder affecting a single organ—the skin. Consequently, current classification schemes divide morphea into categories based solely on cutaneous morphology, without reference to systemic disease or autoimmune phenomena. This classification is likely incomplete.

The prevalence of morphea in childhood is not well established, but reports suggest that approximately two-thirds of linear morphea cases occur before age 18 years, and 20% to 30% of morphea cases overall begin in childhood.^1,2 Studies have described substantial morbidity in children with morphea, but to our knowledge none has addressed the disease effects when the patients reach adulthood (age ≥18 years). To assess the impact of childhood morphea in adults, we searched the Morphea Registry and DNA Repository at the University of Texas Southwestern Medical Center (UTSW) for adults with pediatric-onset morphea (APOMs), and we report our findings herein.

Morphea, also known as localized scleroderma, is characterized by excessive collagen deposition that results in sclerosis of the dermis and sometimes subcutaneous tissue. Morphea causes significant morbidity due to associated functional and cosmetic impairment, reduced quality of life, and rarely, internal manifestations.^1,2 While the pathophysiologic mechanism of morphea is poorly described, it is considered an autoimmune disease, at least partially because of the reported autoantibody associations. Several studies have also reported an association between autoantibodies and disease activity and severity, especially anti–single-stranded DNA antibody (ssDNA ab) in linear morphea.^3-7 However, these studies are limited by lack of controls, small sample size, variable definition of morphea subtypes, different criteria for defining disease activity and/or severity, and the use of different autoantibody assays and cutoff titers. As a result, the prevalence of autoantibodies in morphea remains uncertain, as does the nature of the association between these autoantibodies and disease activity and severity. Nonetheless, our own cross-sectional survey of dermatologists and rheumatologists practicing in the United States revealed that 15% to 47% order ANA testing in the evaluation of their patients with morphea.⁸