TY - JOUR
T1 - Linear morphea
T2 - Clinical characteristics, disease course, and treatment of the Morphea in Adults and Children cohort
AU - Kunzler, Elaine
AU - Florez-Pollack, Stephanie
AU - Teske, Noelle
AU - O'Brien, Jack
AU - Prasad, Smriti
AU - Jacobe, Heidi
N1 - Funding Information:
Funding sources: Supported by the National Center for Advancing Translational Sciences of the National Institutes of Health under award number TL1TR001104. Funding sources: Supported by the National Center for Advancing Translational Sciences of the National Institutes of Health under award number TL1TR001104. We acknowledge Rose Cannon for her contribution to this manuscript's preparation for submission. We also thank the participants of the MAC cohort for their contributions to this study. Funding sources: Supported by the National Center for Advancing Translational Sciences of the National Institutes of Health under award number TL1TR001104.
PY - 2019/6
Y1 - 2019/6
N2 - 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.
AB - 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.
KW - MAC cohort
KW - Morphea in Adults and Children cohort
KW - Parry-Romberg syndrome
KW - en coup de sabre
KW - linear morphea
KW - localized scleroderma
KW - quality of life
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U2 - 10.1016/j.jaad.2019.01.050
DO - 10.1016/j.jaad.2019.01.050
M3 - Article
C2 - 31005342
AN - SCOPUS:85064326757
VL - 80
SP - 1664-1670.e1
JO - Journal of the American Academy of Dermatology
JF - Journal of the American Academy of Dermatology
SN - 0190-9622
IS - 6
ER -