Christen-Zaech S, Hakim MD, Afsar FS, Paller AS
Pediatric morphea (localized scleroderma): Review of 136 patients. [JOURNAL ARTICLE]
J Am Acad Dermatol 2008 Jun 19.
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.
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