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Papillary Dermis

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Linear scleroderma 'en coup de sabre'.

Patxi Zavala Gottau1, Francisco Caiza-Zambrano2, Luis Ariel Miquelini3

  • 1Neurology Department, Hospital Britanico de Buenos Aires, Buenos Aires, Argentina patxizavalagottau@gmail.com.

Practical Neurology
|January 23, 2026
PubMed
Summary
This summary is machine-generated.

Linear scleroderma en coup de sabre, a rare adult-onset condition, can manifest with neurological issues like epilepsy alongside scalp lesions. Early diagnosis and treatment with mycophenolate mofetil can stabilize the condition.

Keywords:
ANTICONVULSANTSCLINICAL NEUROLOGYEPILEPSYIMMUNOLOGYMRI

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Area of Science:

  • Dermatology
  • Neurology
  • Pathology

Background:

  • Linear scleroderma en coup de sabre is a rare inflammatory and fibrotic skin condition.
  • It infrequently occurs in adults and rarely presents with extracutaneous manifestations, predominantly neurological.

Purpose of the Study:

  • To report a case of adult-onset linear scleroderma en coup de sabre with neurological involvement.
  • To highlight the importance of considering this diagnosis in adults presenting with scalp lesions and seizures.

Main Methods:

  • Case presentation of a 43-year-old woman with a forehead lesion and epilepsy.
  • Magnetic Resonance (MR) brain imaging to assess neurological changes.
  • Skin biopsy for histopathological confirmation of scleroderma.

Main Results:

  • The patient exhibited a scar-like forehead lesion and epilepsy.
  • MR brain scan revealed ipsilateral focal cortical atrophy and white matter changes.
  • Skin biopsy confirmed hyaline sclerosis consistent with scleroderma.
  • Clinical stabilization was achieved with mycophenolate mofetil.

Conclusions:

  • Adult-onset linear scleroderma en coup de sabre, though uncommon, should be considered in adults with scalp lesions and epilepsy.
  • Neurological manifestations are significant in adult-onset cases.
  • Mycophenolate mofetil may be an effective treatment option.