Objective:
To describe the neurologic complications and imaging findings in a patient with linear scleroderma en coup de sabre (LScs) and associated Parry-Romberg syndrome.
Background:
LScs is a rare subset of localized scleroderma. Once thought to be an exclusively cutaneous disorder, recent studies suggest that rheumatologic, ophthalmologic, and neurologic symptoms occur in up to 20% of cases.
Design/Methods:
Case report.
Results:
A 32-year-old woman with LScs, associated Parry-Romberg syndrome, migraine, and prior left hemispheric stroke thought to be secondary to vasculitis presented with right arm and leg numbness. She had left hemifacial atrophy. Brain MRI showed an acute ischemic stroke in the posterior limb of the left internal capsule and a chronic T2/FLAIR-hyperintensity in the left frontal lobe immediately beneath the cutaneous forehead and scalp scar. She was previously on mycophenolate for presumed vasculitis, however she had discontinued it due to concern about immunosuppression during the COVID-19 pandemic. Computed tomography angiography, digital subtraction angiography and lumbar puncture were normal. Her stroke was thought to be due to small vessel occlusion. The occurrence of two ischemic strokes ipsilateral to the cutaneous changes and facial atrophy, the ipsilateral MRI findings mirroring the coup de sabre scar, and the occurrence of the new stroke after discontinuation of mycophenolate together suggest an underlying vasculopathy/vasculitis associated with her scleroderma. Her hospital course was complicated by episodes of rapid rhythmic shaking of the right leg with no alteration of awareness, suggesting simple focal motor seizures. She was started on a prednisone with plans for steroid-sparing immunosuppressive therapy (rituximab and mycophenolate). On follow-up evaluation, she was recovering well.
Conclusions:
LScs and Parry-Romberg syndrome are associated with multiple neurologic complications including ischemic strokes, seizures, and headaches. This case highlights the underrecognized relationship between neurologic complications and LScs and suggests an underlying inflammatory process responsive to immunosuppressive therapy.