Risk factors for morphoea disease severity: a retrospective review of 114 paediatric patients

Authors

  • M.S. Pequet,

    1. Creighton University School of Medicine, Omaha, NE, U.S.A
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  • K.E. Holland,

    1. Department of Dermatology, Section of Pediatric Dermatology, Medical College of Wisconsin, Milwaukee, WI, U.S.A
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  • S. Zhao,

    1. Division of Biostatistics, Institute for Health and Society, Medical College of Wisconsin, Milwaukee, WI, U.S.A
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  • B.A. Drolet,

    1. Department of Dermatology, Section of Pediatric Dermatology, Medical College of Wisconsin, Milwaukee, WI, U.S.A
    2. Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI, U.S.A
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  • S.S. Galbraith,

    1. Department of Dermatology, Section of Pediatric Dermatology, Medical College of Wisconsin, Milwaukee, WI, U.S.A
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  • D.H. Siegel,

    1. Department of Dermatology, Section of Pediatric Dermatology, Medical College of Wisconsin, Milwaukee, WI, U.S.A
    2. Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI, U.S.A
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  • Y.E. Chiu

    Corresponding author
    1. Department of Dermatology, Section of Pediatric Dermatology, Medical College of Wisconsin, Milwaukee, WI, U.S.A
    2. Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI, U.S.A
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  • Funding sources This publication was supported by the National Center for Research Resources and the National Center for Advancing Translational Sciences, National Institutes of Health NIH, through Grant Number UL1TR000055. It was also supported, in part, by grant 1UL1RR031973 from the Clinical and Translational Science Award (CTSI) programme of the National Center for Research Resources, National Institutes of Health. Its contents are solely the responsibility of the authors and do not necessarily represent the official views of the NIH.
  • Conflicts of interest None declared

Summary

Background

Morphoea is a rare fibrosing disease of the skin and subcutaneous tissue with an unpredictable disease course, running the spectrum from mild skin involvement to severe disfigurement or extracutaneous complications.

Objectives

Our objective was to describe the natural history of paediatric morphoea and determine patient variables that were associated with severe disease.

Patients and methods

We conducted a retrospective chart review of patients with morphoea seen in one paediatric hospital system. Information about demographics, clinical characteristics, disease course and treatment were collected. Statistical analysis was performed using appropriate univariate tests and a multivariable model.

Results

One hundred and fourteen patients met study inclusion criteria. The female : male ratio was 2·6 : 1, and the median age of onset was 7 years old. There were 55 patients (48%) with linear morphoea, 38 patients (33%) with circumscribed morphoea, 12 patients (11%) with generalized morphoea, and nine patients (8%) with mixed morphoea. Neurological symptoms and joint involvement were present in 27 subjects (24%). Extracutaneous manifestations occurred in 38% of subjects with linear morphoea, compared with 15% with generalized morphoea and 3% with circumscribed morphoea (= 0·0001). Thirty-six per cent of children with disease onset prior to 10 years of age and 5% of children with disease onset after 10 years of age had extracutaneous manifestations (= 0·0002). Both linear morphoea and early-onset disease were significantly associated with extracutaneous involvement in a multivariable model.

Conclusions

Children with linear morphoea and disease onset before 10 years of age should be monitored closely for extracutaneous manifestations and need early treatment with systemic medications to prevent disease complications.

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