Pediatric morphea (localized scleroderma): review of 136 patients

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.