Linear scleroderma en coup de sabre with associated neurologic abnormalities.
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.
Holland
Kristen E
KE
Department of Dermatology, Medical College of Wisconsin, Milwaukee, Wisconsin, USA. kholland@mcw.edu
Steffes
Burt
B
Nocton
James J
JJ
Schwabe
Michael J
MJ
Jacobson
Richard D
RD
Drolet
Beth A
BA
D020330:Bell Palsy / Q000150:complications*; D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D017029:Epilepsy, Complex Partial / Q000150:complications*; D005260:Female; D006801:Humans; D008297:Male; D012536:Scalp Dermatoses / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D012594:Scleroderma, Localized / Q000150:complications* / Q000188:drug therapy / Q000473:pathology