Linear Scleroderma en coup de sabre With Associated Neurologic Abnormalities

Author:

Holland Kristen E.1,Steffes Burt,Nocton James J.2,Schwabe Michael J.3,Jacobson Richard D.3,Drolet Beth A.1

Affiliation:

1. Departments of Pediatrics and Dermatology

2. Department of Pediatrics, Division of Rheumatology

3. Department of Neurology, Division of Pediatric NeurologyMedical College of Wisconsin, Milwaukee, Wisconsin

Abstract

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.

Publisher

American Academy of Pediatrics (AAP)

Subject

Pediatrics, Perinatology and Child Health

Reference34 articles.

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2. Peterson LS, Nelson AM, Su WPD. Classification of morphea (localized scleroderma). Mayo Clin Proc. 1995;70:1068–1076

3. Marzano AV, Menni S, Parodi A, et al. Localized scleroderma in adults and children: clinical and laboratory investigations of 239 cases. Eur J Dermatol. 2003;13:171–176

4. Stone J, Franks AJ, Guthrie JA, Johnson MH. Scleroderma “en coup de sabre”: pathological evidence of intracerebral inflammation. J Neurol Neurosurg Psychiatry. 2001;70:382–385

5. Flores-Alvarado DE, Esquivel-Valerio JA, Garza-Elizondo M, Espinoza LR. Linear scleroderma en coup de sabre and brain calcification: is there a pathologic relationship?J Rheumatol. 2003;30:193–195

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