Clinical Features of Patients with Morphea and the Pansclerotic Subtype: A Cross-sectional Study from the Morphea in Adults and Children Cohort

Abstract

Objective.Pansclerotic morphea is a poorly described form of morphea with little information on prevalence, demographics, and clinical features. Classification criteria for this subtype varies and the distinction from other forms of morphea, such as extensive generalized morphea and pansclerotic morphea, is not always clear. The purpose of our study was to clarify classification criteria for pansclerotic morphea by identifying its prevalence in the morphea in adults and children (MAC) cohort and describing its demographic and clinical features as compared with generalized morphea.Methods.Patients who met predefined criteria for generalized and pansclerotic morphea were identified using a modified Laxer and Zulian classification system. Baseline demographic and clinical features of the patients were compiled and then analyzed for traits characteristic of pansclerotic morphea versus those of generalized morphea. One hundred and thirteen patients met the criteria for inclusion: pansclerotic (n = 13) and generalized morphea type (n = 100).Results.Patients with pansclerotic morphea were more frequently male (46.2% vs 6%; p < 0.0001); had a shorter time to diagnosis (mean difference of 10.4 mos; 95% CI: 0.8–19.9 mos; p = 0.0332); higher rates of functional impairment (61.5% vs 16%; p = 0.0046); higher rates of deep involvement (61.5% vs 17%; p = 0.004); and higher average Rodnan Skin Score (mean difference of 10.8 points; 95% CI: 5–16.6; p = 0.0017), Localized Scleroderma Skin Damage Index (mean difference 28.3; 95% CI: 9–47.6; p = 0.009), and Physician Global Assessment of Disease Damage scores (mean difference 25.1; 95% CI: 0.3–50; p = 0.048).Conclusion.Our results suggest demographic and clinical features are sufficient to define the pansclerotic subtype as they represent a distinct clinical phenotype with a more rapidly progressive and severe course commonly accompanied by disability. Presence of features of the pansclerotic phenotype should alert practitioners to the possibility of significant morbidity and the need for early aggressive treatment.