ArticleOrphanet journal of rare diseases2019
Linear scleroderma "en coup de sabre" with extensive brain involvement-Clinicopathologic correlations and response to anti-Interleukin-6 therapy.
Article in Orphanet journal of rare diseases, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers, 2 of them syntheses that pooled it.
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Who cites it
13 citing papers in PubMed, 2 syntheses or guidelines pooled it, 34 citations in OpenAlex.
- Neurological involvement in localized scleroderma: a systematic review of clinical manifestations and neuroimaging findings.Frontiers in immunology · 2026Pooled it
- Targeting Inflammatory Mediators in Epilepsy: A Systematic Review of Its Molecular Basis and Clinical Applications.Frontiers in neurology · 2022Pooled it
- Successful tocilizumab-based combination therapy for a case of rapidly progressive adult deep morphea with multiple antiphospholipid antibodies: a case report and literature review.Frontiers in immunology · 2026Review
- Long-term follow-up of linear scleroderma en coup de sabre in children with central nervous system involvement.Frontiers in immunology · 2026Article
- Recurrent Seizures in a Case of Linear Scleroderma En Coup de Sabre.Journal of clinical neurology (Seoul, Korea) · 2024Article
- Recent Advances in Treatment of Systemic Sclerosis and Morphea.American journal of clinical dermatology · 2024Review
- Tocilizumab for refractory morphea in adults: A case series.JAAD case reports · 2022Article
- Linear scleroderma in a child with central nervous system involvement: clinical and radiological features.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2022Article
- Therapeutic and Reconstructive Management Options in Scleroderma (Morphea) en Coup de Sabre in Children and Adults. A Systematic Literature Review.Journal of clinical medicine · 2021Review
- Parry Romberg disease with En Coup de Sabre Scleroderma: Effect of tocilizumab on periodontal bone inflammation.Journal of scleroderma and related disorders · 2021Article
- Linear scleroderma en coup de sabre presenting with seizures.Radiology case reports · 2020Article
- Overview of Juvenile localized scleroderma and its management.World journal of pediatrics : WJP · 2020Review
- Linear Scleroderma of the Head - Updates in management of Parry Romberg Syndrome and En coup de sabre: A rapid scoping review across subspecialties.European journal of rheumatology · 2020Article
Corrections and comments
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Authors and funding
5 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Linear scleroderma "en coup de sabre" (LSES) variant is a cephalic subtype of localized scleroderma that can be associated with extracutaneous stigmata, such as epilepsy, dementia syndromes, as well as focal central nervous system neurologic deficits. While the pathophysiology of cutaneous linear scleroderma includes endothelial cell injury and up regulation of pro-fibrogenic pathways, the basis of LSES-associated neurologic complications is largely unknown. We report a patient with a history of LSES who developed intractable epilepsy and cognitive decline. Magnetic resonance imaging (MRI) of the brain exhibited numerous persistently enhancing brain lesions. Due to progressive neurologic deterioration over a period of 7 years, despite interventional therapy, a brain biopsy was performed. Neuropathologic analysis exhibited acute and chronic cortical ischemia associated with a small vessel lymphocytic vasculitis. Direct immunofluorescent studies showed C5b-9 and IgG deposition on endothelium while indirect immunofluorescent studies demonstrated reactivity of the patient's serum with the microvasculature of the patient's own brain tissue and generic human umbilical vein endothelial cells indicative of anti-endothelial cell antibodies. Therapy focusing on damaged endothelium was implemented. The interleukin-6 (IL-6) receptor inhibitor tocilizumab was used and the patient improved dramatically, likely reflecting the drug's effect on the replenishment of endothelial progenitor cells.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.