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CC BY 4.0 · Arq Neuropsiquiatr 2024; 82(12): s00441790574
DOI: 10.1055/s-0044-1790574
Images in Neurology

Multiple cerebral cavernomas in linear scleroderma: an unusual association

Múltiplos cavernomas cerebrais na esclerodermia linear: uma associação incomum
Gabriela Rodrigues Tomaz 
1   Universidade Federal de São Paulo, Escola Paulista de Medicina, Disciplina de Neurologia, Setor de Neurologia Geral e Ataxias, São Paulo SP, Brazil.
,
Maria Eduarda Slhessarenko Fraife Barreto 
1   Universidade Federal de São Paulo, Escola Paulista de Medicina, Disciplina de Neurologia, Setor de Neurologia Geral e Ataxias, São Paulo SP, Brazil.
,
Rafael Tuzino Leite Neves Maffei 
1   Universidade Federal de São Paulo, Escola Paulista de Medicina, Disciplina de Neurologia, Setor de Neurologia Geral e Ataxias, São Paulo SP, Brazil.
,
Renato Barradas Rodrigues 
1   Universidade Federal de São Paulo, Escola Paulista de Medicina, Disciplina de Neurologia, Setor de Neurologia Geral e Ataxias, São Paulo SP, Brazil.
,
Sebastião Boanerges de Araujo Neto 
2   Universidade Federal de São Paulo, Escola Paulista de Medicina, Departamento de Diagnóstico por Imagem, São Paulo SP, Brazil.
,
Marianna Pinheiro Moraes de Moraes 
1   Universidade Federal de São Paulo, Escola Paulista de Medicina, Disciplina de Neurologia, Setor de Neurologia Geral e Ataxias, São Paulo SP, Brazil.
,
José Luiz Pedroso 
1   Universidade Federal de São Paulo, Escola Paulista de Medicina, Disciplina de Neurologia, Setor de Neurologia Geral e Ataxias, São Paulo SP, Brazil.
,
Orlando Graziani Povoas Barsottini 
1   Universidade Federal de São Paulo, Escola Paulista de Medicina, Disciplina de Neurologia, Setor de Neurologia Geral e Ataxias, São Paulo SP, Brazil.
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A 30-year-old female patient presented with recurrent right-sided clonic seizures that had started at the age of 4. Concurrently, her parents noted mild hyperpigmentation, dermal atrophy, and alopecia on the left side of her forehead. She was diagnosed with en coup de sabre scleroderma (ECDS), a subtype of linear scleroderma (LS), given the absence of systemic sclerosis symptoms ([Figure 1]). A brain magnetic resonance imaging (MRI) revealed cavernomas and white matter lesions in the left cerebral hemisphere, ipsilateral to the cutaneous lesions ([Figure 2]).

Zoom Image
Figure 1 Autoimmune atrophy of the skin and subcutaneous tissue promotes the hallmark of the disease - the coup de sabre lesion (A). Three-dimensional reconstruction in volume rendering using brain magnetic resonance imaging (B) additionally demonstrates another coup de sabre in the interparietal region.
Zoom Image
Figure 2 Fluid-attenuated inversion recovery images (A and B) and ADC map (C) shows T2 hypersignal and facilitated diffusion in the subcortical white matter ipsilateral to the skin lesion. Susceptibility-weighted imaging (SWI) (D), SWI phase map (E) and postcontrast T1 spoiled gradient-recalled (SPGR) (F): small subcortical nodules exhibiting blooming on SWI, compatible with cavernomas.

Localized facial scleroderma can be associated with neurological complications, as illustrated by our case involving ipsilateral brain cavernomas.[1] [2] [3] This diagnosis is presumptive, as histopathological confirmation of the lesions was not obtained. Differential diagnoses should consider small hemorrhages, potentially secondary to vasculopathy, as suggested by the susceptibility-weighted imaging (SWI) MRI findings.[4] [5]

Editor-in-Chief

Ayrton Roberto Massaro.


Associate Editor

Leandro Tavares Lucato.


Author's Contributions

GRT, MESFB, RTNLM, RBR: conceptualization, resources, and writing – original draft; SBAN, supervision; MPMM: supervision and writing – review & editing; JLP, OGPB: supervision.




Publication History

Received: 27 May 2024

Accepted: 21 July 2024

Article published online:
24 September 2024

© 2024. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution 4.0 International License, permitting copying and reproduction so long as the original work is given appropriate credit (https://creativecommons.org/licenses/by/4.0/)

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Bibliographical Record
Gabriela Rodrigues Tomaz, Maria Eduarda Slhessarenko Fraife Barreto, Rafael Tuzino Leite Neves Maffei, Renato Barradas Rodrigues, Sebastião Boanerges de Araujo Neto, Marianna Pinheiro Moraes de Moraes, José Luiz Pedroso, Orlando Graziani Povoas Barsottini. Multiple cerebral cavernomas in linear scleroderma: an unusual association. Arq Neuropsiquiatr 2024; 82: s00441790574.
DOI: 10.1055/s-0044-1790574
 

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