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

Emily T Fain,Ronald M Laxer,Randy Q Cron,Daniel W Young,Elena Pope,Melissa Mannion

doi:10.1186/1546-0096-9-18

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.

Highlights

Localized scleroderma encompasses the conditions of linear scleroderma (LS), plaque or circumscribed morphea, pansclerotic and generalized morphea
A literature review done by Kister et al found that the skin lesion preceded the onset of neurological symptoms by an average of several years, in 29% of the patients studied the two occurred within one year of each other [7]
We report the first associations with ipsilateral central nervous system (CNS) cavernomas

Summary

Background

Localized scleroderma encompasses the conditions of linear scleroderma (LS) (extremity and facial), plaque or circumscribed morphea, pansclerotic and generalized morphea. Case Presentation A patient with LS en coup de sabre at the Children’s Hospital of Alabama was noted to have brain cavernomas by MRI. At initial evaluation 9 months later, an en coup de sabre lesion was noted near the midline on right side of the face, extending from nose to hairline and had become sunken per the family. Since the lesion appeared to be getting worse and headaches and seizures were ongoing, the patient was initially treated with prednisone 40 mg twice daily for one month, tapered over the course of 2-3 months, and methotrexate 25 mg injected subcutaneously each week. Over the course of the year the patient remained on methotrexate, with doses ranging from 12.5 to 25 mg weekly On this treatment, the skin lesion improved Her neurologic examination was normal and there were no cognitive deficits

Findings

Discussion

Conclusions