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

Elaine Kunzler, Stephanie Florez-Pollack, Noelle Teske, Jack O'Brien, Smriti Prasad, Heidi Jacobe

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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.

Original languageEnglish (US)
JournalJournal of the American Academy of Dermatology
DOIs
Publication statusPublished - Jan 1 2019

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Keywords

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

ASJC Scopus subject areas

  • Dermatology

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