Elaine Kunzler1, Stephanie Florez-Pollack2, Noelle Teske2, Jack O'Brien2, Smriti Prasad2, Heidi Jacobe3. 1. Department of Dermatology, University of Texas Southwestern Medical Center, Dallas, Texas; Northeast Ohio Medical University, Rootstown, Ohio. 2. Department of Dermatology, University of Texas Southwestern Medical Center, Dallas, Texas. 3. Department of Dermatology, University of Texas Southwestern Medical Center, Dallas, Texas. Electronic address: heidi.jacobe@utsouthwestern.edu.
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.
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
Authors: Justyna Szczęch; Dominik Samotij; Kamila Jaworecka; Aleksandra Tobiasz; Adam Reich Journal: Biomed Res Int Date: 2020-03-13 Impact factor: 3.411