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Linear scleroderma "en coup de sabre": initial presentation as intractable partial seizures in a child
Kuo-Liang Chiang1, Kai-Ping Chang, Tai-Tong Wong
1Department of Pediatrics, Neurological Institute, Taipei Veterans General Hospital, Taipei, Taiwan.
Pediatrics and Neonatology
|December 23, 2009
Summary
Linear scleroderma en coup de sabre (LSCS) can present with neurological issues like seizures before skin lesions appear. This rare case highlights brain involvement in LSCS, emphasizing the need for differential diagnosis with Parry-Romberg syndrome.
Area of Science:
- Neurology
- Dermatology
- Radiology
Background:
- Linear scleroderma en coup de sabre (LSCS) is a localized scleroderma subtype.
- LSCS is associated with systemic complications, including neurological abnormalities.
Observation:
- A patient presented with refractory partial seizures preceding characteristic skin lesions.
- Brain imaging revealed significant intraparenchymal involvement, a rare presentation of LSCS.
Findings:
- The patient's seizures and brain parenchymal lesion remained stable without medication.
- Atrophic changes in the skin, face, and brain showed no progression.
Implications:
- This case underscores the importance of considering neurological symptoms in LSCS diagnosis.
- Differentiating LSCS from Parry-Romberg syndrome is crucial due to overlapping features.
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