Pediatric morphea (localized scleroderma): Review of 136 patients - 24/04/13
Abstract |
Background |
Morphea is an autoimmune inflammatory sclerosing disorder that may cause permanent functional disability and disfigurement.
Objectives |
We sought to determine the clinical features of morphea in a large pediatric cohort.
Methods |
We conducted a retrospective chart review of 136 pediatric patients with morphea from one center, 1989 to 2006.
Results |
Most children showed linear morphea, with a disproportionately high number of Caucasian and female patients. Two patients with rapidly progressing generalized or extensive linear morphea and arthralgias developed restrictive pulmonary disease. Initial oral corticosteroid treatment and long-term methotrexate administration stabilized and/or led to disease improvement in most patients with aggressive disease.
Limitations |
Retrospective analysis, relatively small sample size, and risk of a selected referral population to the single site are limitations.
Conclusions |
These data suggest an increased prevalence of morphea in Caucasian girls, and support methotrexate as treatment for problematic forms. Visceral manifestations rarely occur; the presence of progressive problematic cutaneous disease and arthralgias should trigger closer patient monitoring.
Le texte complet de cet article est disponible en PDF.Abbreviations used : ANA, ECDS, ESR, PHA, UV
Plan
Funding sources: None. |
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Conflicts of interest: None declared. |
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Reprints not available from the authors. |
Vol 59 - N° 3
P. 385-396 - septembre 2008 Retour au numéroBienvenue sur EM-consulte, la référence des professionnels de santé.
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