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.
Risk factors for morphoea disease severity: a retrospective review of 114 paediatric patients
Article first published online: 15 APR 2014
© 2013 British Association of Dermatologists
British Journal of Dermatology
Volume 170, Issue 4, pages 895–900, April 2014
How to Cite
Pequet, M.S., Holland, K.E., Zhao, S., Drolet, B.A., Galbraith, S.S., Siegel, D.H. and Chiu, Y.E. (2014), Risk factors for morphoea disease severity: a retrospective review of 114 paediatric patients. British Journal of Dermatology, 170: 895–900. doi: 10.1111/bjd.12758
Conflicts of interest None declared
- Issue published online: 15 APR 2014
- Article first published online: 15 APR 2014
- Accepted manuscript online: 17 DEC 2013 12:21AM EST
- Manuscript Accepted: 23 NOV 2013
- National Center for Research Resources
- National Center for Advancing Translational Sciences
- National Institutes of Health NIH. Grant Number: UL1TR000055
- Clinical and Translational Science Award (CTSI). Grant Number: 1UL1RR031973
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.
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.
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 (P = 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 (P = 0·0002). Both linear morphoea and early-onset disease were significantly associated with extracutaneous involvement in a multivariable model.
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.