Linear morphea: Clinical characteristics, disease course, and treatment of the Morphea in Adults and Children cohort

Abstract

Background: Prospective, longitudinal studies examining the features of linear morphea are limited.

Objective: To utilize the Morphea in Adults and Children cohort to determine clinical characteristics, impact on life quality, and disease course of linear morphea in a prospective, longitudinal manner.

Methods: Characteristics of linear morphea versus other subtypes were compared in a cross-sectional manner. Next, linear morphea participants were examined in depth over a 3-year period.

Results: Linear morphea was the most common morphea subtype (50.1%, 291/581) in the cohort. Deep involvement was more common in linear (64.3%, 187/291) than other morphea subtypes. Linear morphea participants with deep involvement were more likely to have a limitation in range of motion (28.6%, 55/192) than those without (11.1%, 11/99, P < .001). Adult-onset disease occurred in 32.6% (95/291) of those with linear morphea. Frequency of deep involvement was similar between pediatric (66.8%, 131/196) and adult-onset linear morphea (58.9%, 56/95, P = .19). Quality of life and disease activity scores improved over time, while damage stabilized with treatment.

Limitations: Results of the study are associative, and the University of Texas Southwestern Medical Center is a tertiary referral center.

Conclusion: A substantial number of linear morphea patients have adult-onset disease. In all age groups, linear morphea with deep involvement was associated with functional limitations.

Keywords: MAC cohort; Morphea in Adults and Children cohort; Parry-Romberg syndrome; en coup de sabre; linear morphea; localized scleroderma; quality of life.