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.
Publisher Copyright:
© 2019 American Academy of Dermatology, Inc.
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
UR - http://www.scopus.com/inward/record.url?scp=85064326757&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85064326757&partnerID=8YFLogxK
U2 - 10.1016/j.jaad.2019.01.050
DO - 10.1016/j.jaad.2019.01.050
M3 - Article
C2 - 31005342
AN - SCOPUS:85064326757
SN - 0190-9622
VL - 80
SP - 1664-1670.e1
JO - Journal of the American Academy of Dermatology
JF - Journal of the American Academy of Dermatology
IS - 6
ER -