Pediatric morphea (localized scleroderma): Review of 136 patients

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.

Abbreviations used: ANA, antinuclear antibody, ECDS, en coup de sabre, ESR, erythrocyte sedimentation rate, PHA, progressive hemifacial atrophy, UV, ultraviolet