TY - JOUR
T1 - Pediatric morphea (localized scleroderma)
T2 - Review of 136 patients
AU - Christen-Zaech, Stéphanie
AU - Hakim, Miriam D.
AU - Afsar, F. Sule
AU - Paller, Amy S.
PY - 2008/9
Y1 - 2008/9
N2 - 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.
AB - 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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U2 - 10.1016/j.jaad.2008.05.005
DO - 10.1016/j.jaad.2008.05.005
M3 - Article
C2 - 18571769
AN - SCOPUS:48749115529
SN - 0190-9622
VL - 59
SP - 385
EP - 396
JO - Journal of the American Academy of Dermatology
JF - Journal of the American Academy of Dermatology
IS - 3
ER -