Linear scleroderma ‘en coup de sabre’ associated with cerebral and ocular vasculitis

Abstract

Linear scleroderma 'en coup de sabre' (LSCS) has been reported in association with intracranial abnormalities. We report the case of an 11-year-old boy with LSCS who presented with recurrent headaches. Cranial magnetic resonance imaging (MRI) and angiography were consistent with the diagnosis of a cerebral vasculitis. In addition, retinal examination showed an exudative inflammatory lesion consistent with vasculitis.

Ocular involvement in children with localised scleroderma: a multi-centre study

“…The reported alterations, as in our study, may involve adnexa structures,3 the anterior or posterior segments of the eye4 6 7 and the CNS 5 8 – 10. The involvement of the extrinsic eye muscles, as in one of our patients (no.19), is extremely rare but well described by both CT and MRI4 13 and should be suspected in presence of pain on eye movement, diplopia and strabismus.…”

Esclerodermia localizada na criança: aspectos clínicos, diagnósticos e terapêuticos

“…30,31,32,33 Os achados imagenológicos (RNM, SPECT) incluem atrofia ou calcificações corticais. Análise liquórica é compatível com inflamação intracerebral (Figura 3).…”

Diagnostic criteria, severity classification and guidelines of localized scleroderma

“…153,161 There have been case reports in which corticosteroids, methotrexate and mycophenolate mofetil effectively treated recurrent headaches and cranial nerve lesions. 142,143,162,163 There have also been reports indicating that anticonvulsants and antidepressants effectively mitigated symptoms. 144,164,165 One case report indicated that corticosteroids and azathioprine were ineffective for treating optic papillitis.…”

“…145 One case of cerebral vasculitis, which improved with mycophenolate, has been reported. 162 Based on the above information, antiepileptic drugs are effective for treating mild epileptic seizures that are caused by brain lesions, while combined systemic steroid therapy and immunosuppressants is considered to be effective for moderate or worse epilepsy, including generalized tonic clonic seizures or treatment-resistant epileptic seizures. The evidence level on antiepileptic drugs is low, but the recommendation level is set as 1D, based on the consensus of the committee that created this guideline.…”