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Pediatric morphea (localized scleroderma): review of 136 patients
Stéphanie Christen-Zaech1, Miriam D Hakim, F Sule Afsar
1Department of Dermatology, Northwestern University Feinberg School of Medicine, Chicago, Illinois 60611-2997, USA.
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.
Insights
Morphea, an autoimmune skin condition, is more common in Caucasian girls. Methotrexate is effective for severe cases, though visceral issues are rare. Close monitoring is advised for progressive disease.
Area of Science:
- Dermatology
- Autoimmune Disorders
- Pediatrics
Background:
- Morphea is an autoimmune inflammatory sclerosing disorder.
- It can lead to functional disability and disfigurement.
Purpose of the Study:
- To determine the clinical features of morphea in a large pediatric cohort.
- To identify risk factors and treatment outcomes in pediatric morphea.
Main Methods:
- Retrospective chart review of 136 pediatric patients.
- Data collected from 1989 to 2006 at a single center.
Main Results:
- Linear morphea was most common, particularly in Caucasian females.
- Two patients developed restrictive pulmonary disease due to aggressive morphea with arthralgias.
- Corticosteroids and methotrexate improved or stabilized disease in most aggressive cases.
Conclusions:
- Morphea appears more prevalent in Caucasian girls.
- Methotrexate is a viable treatment for problematic morphea forms.
- Visceral involvement is rare, but progressive cutaneous disease and arthralgias warrant vigilant monitoring.
