Pediatrics & Neonatology
Volume 50, Issue 6 , Pages 294-298, December 2009

Linear Scleroderma “En Coup De Sabre”: Initial Presentation as Intractable Partial Seizures in a Child

  • Kuo-Liang Chiang

      Affiliations

    • Department of Pediatrics, Neurological Institute, Taipei Veterans General Hospital, Taipei, Taiwan
    • Department of Pediatrics, Kuang-Tien General Hospital, Taichung, Taiwan
    • Department of Neurosurgery, Neurological Institute, Taipei Veterans General Hospital, Taipei, Taiwan
    • ,
  • Kai-Ping Chang

      Affiliations

    • Department of Pediatrics, Neurological Institute, Taipei Veterans General Hospital, Taipei, Taiwan
    • Corresponding Author InformationCorresponding author. Department of Pediatrics, Taipei Veterans General Hospital, 201 Shih-Pai Road, Section 2, Taipei 112, Taiwan
    • ,
  • Tai-Tong Wong

      Affiliations

    • Department of Pediatrics, Kuang-Tien General Hospital, Taichung, Taiwan
    • Department of Neurosurgery, Neurological Institute, Taipei Veterans General Hospital, Taipei, Taiwan
    • National Yang-Ming University School of Medicine, Taipei, Taiwan
    • ,
  • Ting-Rong Hsu

      Affiliations

    • Department of Pediatrics, Neurological Institute, Taipei Veterans General Hospital, Taipei, Taiwan
    • National Yang-Ming University School of Medicine, Taipei, Taiwan

Received 22 January 2009; received in revised form 18 May 2009; accepted 25 May 2009.

Article Outline

Linear scleroderma is a form of localized scleroderma characterized by sclerotic lesions distributed in a linear, band-like pattern. The “en coup de sabre” subtype of linear scleroderma is more often associated with systemic morbidity, including ocular, oral, and neurological abnormalities. Here, we report one patient with typical linear scleroderma “en coup de sabre” (LSCS). Initially, he presented with refractory partial seizures before the characteristic skins lesion on his head developed. This was a rare case with obvious brain parenchyma involvement. We did not prescribe medication but performed serial brain magnetic resonance imaging follow-up for the intraparenchymal lesion. The atrophic changes of the skin, face and brain remained the same, and his seizures had not worsened at the most recent follow-up. Parry-Romberg syndrome, a very similar condition, should be differentiated from LSCS.

Key Words:  intractable seizure , linear scleroderma en coup de sabre , progressive brain atrophy

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PII: S1875-9572(09)60081-4

doi:10.1016/S1875-9572(09)60081-4

Pediatrics & Neonatology
Volume 50, Issue 6 , Pages 294-298, December 2009

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