Pediatric morphea (localized scleroderma): review of 136 patients.
BACKGROUND
Morphea is an autoimmune inflammatory sclerosing disorder that may cause permanent functional disability and disfigurement.


OBJECTIVE
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.


CONCLUSIONS
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.
Christen-Zaech
Stéphanie
S
Department of Dermatology, Northwestern University Feinberg School of Medicine, Chicago, Illinois 60611-2997, USA.
Hakim
Miriam D
MD
Afsar
F Sule
FS
Paller
Amy S
AS
D000293:Adolescent; D017668:Age of Onset; D000974:Antibodies, Antinuclear / Q000097:blood; D001327:Autoimmune Diseases / Q000150:complications; D002648:Child; D002675:Child, Preschool; D003879:Dermatologic Agents / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008727:Methotrexate / Q000627:therapeutic use*; D015995:Prevalence; D012189:Retrospective Studies; D012307:Risk Factors; D012594:Scleroderma, Localized / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy* / Q000208:ethnology; D012595:Scleroderma, Systemic / Q000150:complications; D012737:Sex Factors; D012867:Skin / Q000473:pathology; D044465:White People
D000974:Antibodies, Antinuclear; D003879:Dermatologic Agents; D008727:Methotrexate