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Journal of the American Academy of Dermatology
Volume 80, n° 6
page 1664 (juin 2019)
Doi : 10.1016/j.jaad.2019.01.050
accepted : 21 January 2019
Original Articles

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

Elaine Kunzler, MD a, b, Stephanie Florez-Pollack, BS a, Noelle Teske, MD, MSc a, Jack O'Brien, MD a, Smriti Prasad, BS a, Heidi Jacobe, MD, MSCS a,
a Department of Dermatology, University of Texas Southwestern Medical Center, Dallas, Texas 
b Northeast Ohio Medical University, Rootstown, Ohio 

Correspondence to: Heidi Jacobe, MD, MSCS, Department of Dermatology, UT Southwestern Medical Center, 5323 Harry Hines Blvd., Dallas, TX 75390-9069.Department of DermatologyUT Southwestern Medical Center5323 Harry Hines Blvd.DallasTX75390-9069
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.

The full text of this article is available in PDF format.

Key words : en coup de sabre, linear morphea, localized scleroderma, MAC cohort, Morphea in Adults and Children cohort, Parry-Romberg syndrome, quality of life

Abbreviations used : CDLQI, DLQI, IQR, MAC, LoSCAT, mLoSSI, LoSDI, PGA-A, PGA-D



 Dr Kunzler and Ms Florez-Pollack are co-first authors.
 Funding sources: Supported by the National Center for Advancing Translational Sciences of the National Institutes of Health under award number TL1TR001104.
 Conflicts of interest: None disclosed.
 Disclaimer: The content is solely the responsibility of the authors and does not necessarily represent the official views of the University of Texas Southwestern Medical Center at Dallas or its affiliated academic and health care centers or the National Institutes of Health.
 Reprints not available from the authors.



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