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Neurologic involvement in scleroderma en coup de sabre.

Tiago Nardi Amaral1, João Francisco Marques Neto, Aline Tamires Lapa

  • 1Rheumatology Division, Faculty of Medical Science, State University of Campinas, 13083-970 Campinas, SP, Brazil.

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Localized scleroderma en coup de sabre (LScs) can affect deeper tissues and the nervous system, not just the skin. This study details the clinical and imaging findings of neurological complications in LScs patients.

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Area of Science:

  • Neurology
  • Dermatology
  • Radiology

Background:

  • Localized scleroderma is a rare condition causing skin hardening.
  • Scleroderma en coup de sabre (LScs) presents with characteristic facial lesions.
  • While primarily cutaneous, LScs can involve deeper tissues and the central nervous system.

Purpose of the Study:

  • To describe the clinical manifestations and radiologic findings of neurologic involvement in scleroderma en coup de sabre.
  • To enhance understanding of the differential diagnosis between LScs and Parry-Romberg syndrome.
  • To highlight the importance of neuroimaging in LScs.

Main Methods:

  • Review of clinical cases with LScs and documented neurologic symptoms.
  • Analysis of neuroimaging data including CT, MRI, and cerebral angiograms.
  • Correlation of clinical presentation with radiologic findings.

Main Results:

  • Neurologic involvement in LScs includes seizures, focal deficits, movement disorders, and trigeminal neuralgia.
  • Neuroimaging reveals central nervous system lesions consistent with vasculitis.
  • LScs with neurologic involvement presents diagnostic challenges, mimicking other conditions.

Conclusions:

  • Neurologic complications are a significant feature of scleroderma en coup de sabre.
  • Neuroimaging is crucial for diagnosing and characterizing CNS involvement in LScs.
  • Early recognition and management of neurologic symptoms are essential for LScs patients.