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Linear scleroderma en coup de sabre with associated neurologic abnormalities. Pediatrics 2006 Jan;117(1):e132-6



Pubmed ID




Scopus ID

2-s2.0-33644835927   102 Citations


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.

Author List

Holland KE, Steffes B, Nocton JJ, Schwabe MJ, Jacobson RD, Drolet BA


Kristen E. Holland MD Associate Professor in the Dermatology department at Medical College of Wisconsin
James J. Nocton MD Professor in the Pediatrics department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

Bell Palsy
Child, Preschool
Disease Progression
Epilepsy, Complex Partial
Scalp Dermatoses
Scleroderma, Localized