Published online December 1, 2005
PEDIATRICS Vol. 117 No. 1 January 2006, pp. e132-e136 (doi:10.1542/peds.2005-0470)
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ARTICLE

Linear Scleroderma en coup de sabre With Associated Neurologic Abnormalities

Kristen E. Holland, MDa, Burt Steffes, MD, James J. Nocton, MDb, Michael J. Schwabe, MDc, Richard D. Jacobson, MD, PhDc and Beth A. Drolet, MDa

a Departments of Pediatrics and Dermatology
b Department of Pediatrics, Division of Rheumatology
c Department of Neurology, Division of Pediatric NeurologyMedical College of Wisconsin, Milwaukee, Wisconsin

Linear scleroderma represents a unique form of localized scleroderma that primarily affects the pediatric population, with 67% of patients diagnosed before 18 years of age. When linear scleroderma occurs on the head, it is referred to as linear scleroderma en coup de sabre, given the resemblance of the skin lesions to the stroke of a sabre. Here we describe 3 pediatric patients with linear scleroderma en coup de sabre who presented with neurologic abnormalities before or concurrent with the diagnosis of their skin disease. Our patients' cases highlight the underrecognized relationship between neurologic complications and linear scleroderma en coup de sabre and illustrate the importance of a thorough skin examination in patients with unexplained neurologic disease.

Key Words: pediatric • scleroderma • en coup de sabre • neurologic • children

Abbreviations: EEG, electroencephalogram • CT, computed tomography • ANA, antinuclear antibodies

Accepted Jul 22, 2005.


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