Brain cavernomas associated with en coup de sabre linear scleroderma: Two case reports

Abstract

Linear scleroderma is a form of localized scleroderma that primarily affects the pediatric population. When it occurs on the scalp or forehead, it is termed "en coup de sabre". In the en coup de sabre subtype, many extracutaneous associations, mostly neurological, have been described. A patient with linear scleroderma en coup de sabre was noted to have ipsilateral brain cavernomas by magnetic resonance imaging. Using a worldwide pediatric rheumatology electronic list-serve, another patient with the same 2 conditions was identified. These two patients are reported in this study. Consideration of neuroimaging studies to disclose abnormal findings in patients with linear scleroderma en coup de sabre is important for potentially preventing and treating neurological manifestations associated with this condition.

CNS imaging findings associated with Parry–Romberg syndrome and en coup de sabre: correlation to dermatologic and neurologic abnormalities

“…For example, there are previous case reports of cavernomas in association with PRS and ECS [37], but we did not identify a single definite example. Since cavernomas frequently are detected as incidental findings in the pediatric population [38], this association may be coincidental.…”

Neurologic Involvement in Scleroderma en Coup de Sabre

“…Intraparenchymal calcifications involving basal ganglia, thalami, and dentate nuclei are the most common brain lesion in LScs patients [30, 47, 48]. Characteristically, the calcifications are ipsilateral [46, 49], but contralateral involvement may occur [36, 50].…”

Diagnostic criteria, severity classification and guidelines of localized scleroderma

“…Explanation: Epilepsy is the most frequently seen neurological symptom of localized scleroderma, and various antiepileptic drugs are used to treat epileptic seizures. [141][142][143][144][145][146][147][148][149][150][151] The drugs include carbamazepine, oxcarbazepine, phenobarbital, sodium valproate, topiramate, clobazam, pregabalin, nitrazepam, vigabatrin, sultiame and lamotrigine. Antiepileptic drugs were reported to be effective at controlling epileptic seizures in 78% of mild cases.…”

“…The most frequently used drugs are corticosteroids, used in 80% of previously reported cases with a reported 90% efficacy. 127,142,143,[145][146][147]153,154 However, only one case has been treated with corticosteroid monotherapy, 111 while four were treated with steroids and methotrexate, 142,143,146,154 three were treated with corticosteroids combined with azathioprine, 125,129,131 two were treated with corticosteroids combined with cyclophosphamide, 147 and one each were treated with corticosteroids combined with mycophenolate mofetil, 142 IFN-c 145 or high-dose Ig i.v. therapy.…”