Characterizing Morphea Subsets Using a Multi-Center, Prospective, Cross-Sectional Analysis of Morphea in Adults and Children

BACKGROUND: There are few prospective studies in morphea. Most that exist focus exclusively on adults or children, or are insufficiently powered. Others are retrospective, which have limitations in understanding subsets. As a result, there is limited understanding of the demographic and clinical features of morphea, particularly of the less frequent subtypes. There is also little known about the differences between adults and children. OBJECTIVE: To determine the clinical and demographic features of the inception cohort of two prospectively-collected datasets and to determine novel latent disease phenotypes among this cohort. METHODS: This is a cross sectional analysis of initial visits of the Morphea in Adults and Children (MAC) and the National Registry of Childhood-Onset Scleroderma (NRCOS) cohorts, two prospectively collected databases that collect demographic, physical exam and clinical data. We performed traditional univariate analyses as well as multivariate analyses, include a principal component analysis (PCA) of certain variables. RESULTS: Of the total 944 participants, 500 (53%) had pediatric onset morphea, and 444 (47%) had adult onset morphea. Whites (76%) and females (78%) comprised the majority of participants. The median age at onset was 16 years overall. Five factors were extracted from the PCA based on the inflection point in the scree plot (CF1-5). CF2 described a clustering of patients with depression and high comorbidities of headaches, joint pain, muscle pain, and fatigue. Based on this, we did a post-hoc analysis on the quality of life measures collected in adults, which was derived from the SKINDEX 29+3. CONCLUSION: We've described the clinical and demographic features in the largest cohort of morphea patients to date. Here we describe subsets of patients that have been previously described, such as a group of patients with depression and somatic symptoms.