Linear scleroderma en coup de sabre presenting with seizures

• Published in: Radiology case reports 2020-11, Vol.15 (11), p.2164-2170
• Main Authors: Nguyen, Kevin, Atty, Corrine, Ree, Alexander
• Format: Article
• Language: English
• Subjects: Case Report; En coup de sabre; Morphea; MR perfusion; MRI; Scleroderma; Spectroscopy
• ISSN: 1930-0433; 1930-0433
• DOI: 10.1016/j.radcr.2020.08.011

Scleroderma is a rare connective tissue disorder categorized into systemic sclerosis and localized scleroderma, also called morphea. Linear scleroderma of the scalp, also called en coup de sabre, is infrequently associated with neurologic symptoms. We describe a case of linear scleroderma en coup de sabre in a 28-year-old female presenting with seizures and characteristic cutaneous lesions. Imaging findings over a course of 7 years demonstrated waxing and waning signal changes. MR perfusion and spectroscopic imaging, demonstrating decreased cerebral blood volume, increased mean transit time, and decreased metabolites, was performed during a time of progressing radiological and clinical findings. Comparison with other reports in the literature supported several clinical and imaging findings that while not pathognomic, highly suggest the diagnosis of linear scleroderma en coup de sabre. Hyperintense signal on T2W magentic resonance imaging and contrast enhancement on computed tomography and magnetic resonance imaging have been the most commonly described imaging findings. To our knowledge, no previous description of spectroscopic or perfusion imaging of linear scleroderma en coup de sabre have been reported. It is our hope that this report may add MRS and magnetic resonance perfusion findings to a growing knowledge of this rare entity.